COLUMBIA LIBRARIES OFFSITE HEALTH SCIENCES STANDARD ^ 'i 1^c^?^ "hx'b • '. - i :^J«*-' -• * ■ £f /> / ■' CHOREA ON CHOREA AND OTHER ALLIED MOVEMENT DISORDERS OF EARLY LIFE BY OCTAVIUS STURGES, M.D. FELLOW OF THE ROYAL COLLEGE OF PHYSICIANS PHYSICIAN TO THE WESTMINSTER HOSPITAL ASSISTANT-PHYSICIAN TO THE HOSPITAL FOR SICK CHILDREN LONDON SMITH, ELDER, & CO., 15 WATERLOO PLACE 1881 iAll rights reserved'^ TO D^ JOHN ABERCROMBIE MEDICAL REGISTRAR TO THE HOSPITAL FOR SICK CHILDREN '§^15 '^oxk is gnscribeb IN RECOGNITION OF THE SOURCE WHENCE MUCH OF ITS MATERIAL IS DERIVED WITH THE GOOD WISHES OF HIS FELLOW-LABOURER THE AUTHOR PREFACE The object of this book, a portion of which has already appeared in medical journals, is to place before the reader such an account of Chorea, and of the theories which prevail concerning it, as shall enable him to form some judgment as to the nature and suitable treatment of a disorder which is not very common in ordinary practice, yet subject to so many varieties that the study of a few examples gives no adequate conception of its true cha- racter. The first and chief part of the work is occupied with a description of Chorea and of the several hypotheses which have been provided to explain it ; the rest is devoted to an examination of these theories in the light of admitted facts, together with an account of that particular view of the pathology and management of the affection which a full consideration of its phenomena seems to suggest. The Appendices, referring to a large number of cases which have been under my own observation and treatment, claim to furnish some of the evidence upon which the conclusions of the book are founded. In expressing dissent from all anatomical explanations of Chorea, it has been necessary to refer to many eminent vi PREFACE. authorities who have attempted to reach its structural basis. In so doing I must not be understood as setting up my own opinion against accepted doctrines of pathology. The failure, or at least the ambiguity, of morbid anatomy in this particular is not denied, and in calling attention to it I have only given utterance to what is generally ad- mitted. If it should be thought that too much space is occupied with such controversy, the apology must be that the subject in its present stage cannot be otherwise dealt with, and that it would be absurd to discuss the treatment of Chorea until we have come to some agreement as to its nature. My purpose throughout has been to show that Chorea, no less than the many smaller movement transgressions which resemble it, is a disease of function due in large measure to preventible causes ; that the nature and cir- cumstances of children render them apt subjects for such a disorder ; and that, while it is beyond reasonable ex- pectation that the course of childhood should be kept altogether out of its reach, we are not without guidance as to the sort of training and management which affords the surest protection against its attacks, and the best material for its cure. 85 WiMPOLE Street : October 16, 188 1. CONTENTS. INTRODUCTION. PAGE The Place in Nature of Functional Nervous Disease . . . . . i CHAPTER L GENERAL DESCRIPTION OF CHOREA. Pedigree — Definition — Parts affected — Degrees of severity — Mental involve- ment — Temperament — ^Age — Sex — Adult and senile chorea — Geographical distribution — Causes — ^Varieties — Progress — Paralytic symptoms — Re- currence — Connection with heart disorder — Sequelae . . . . ii CHAPTER II. CONNECTION OF RHEUMATISM WITH CHOREA. Analysis of 177 cases — Comparison with other statistics — Conclusions in reference to rheumatic connection, (i) from statistics, (2) from general considerations 32 CHAPTER III. THE HEART SYMPTOMS OF CHOREA. Facts for explanation — The comparative frequency of heart disturbance in chorea of different ages — The heart symptoms referred to endocarditis — to a sympathy on the part of the heart with the muscular disorder — Difficulties in the way of both hypotheses — Conclusions .... 44 a viii CONTENTS. CHAPTER IV. CHOREA AS A FATAL DISEASE. PAGE Recorded examples of fatal chorea — Age and sex in relation to fatality — Exciting causes — Morbid anatomy — Conclusions 72 CHAPTER V. THE PATHOLOGIES OF CHOREA. Connection with rheumatism — with spinal meningitis — with pericarditis — with enfeebled nutrition — The embolic theory of chorea — Vascular and nervous changes — Disturbed nutrition of cerebral ganglia — Altered states of blood — Chorea a functional disturbance — Continental opinion — Sum- mary ............. 82 CHAPTER VI. THE PATHOLOGIES OF CHOREA IN THEIR APPLICATION. Clinical conditions to be satisfied — Symptoms of embolism compared with those of chorea — The direct evidence in favour of the embolic theory — Peri- vascular degeneration of brain and cord as a cause of chorea — Hypothetical explanations — Chorea ascribed to temporary trophic changes — Application of such theories — The ataxy of chorea 96 CHAPTER VII. CHOREA A FUNCTIONAL DISEASE. The parts selected by chorea — Its varieties and modifications — Its form in relation to its cause — Alliance to hysteria and emotion — Manner of pro- gress and of recurrence no CHAPTER VIII. CHOREA A FUNCTIONAL DISEASE {c07ltinucd). Some objections considered — The duration of chorea in relation to its cause — Duration at various ages — Chorea in association with structural lesions — The effects of chorea to be distinguished from its cause — Chorea incom- patible with structural disiase — Various modes of expressing existing knowledge in reference to the pathology of chorea 124 CONTENTS. IX CHAPTER IX. THE TREATMENT OF CHOREA. PAGE Indications to be fulfilled — Removal of source of irritation in young children — Rest — Signs of debility as indications for treatment — Change of place — Drug remedies — Treatment of acute chorea — Chronic chorea — Conva- lescence • 13^ CHAPTER X. THE PREVENTION OF CHOREA. Development and direction of childish movement — Allowance for emotional movement — The therapeutics and morals of movement .... 142 APPENDIX A. Abstracts of Cases illustrating some of the Associations of Chorea :— 1. Heart disturbance preceding chorea 151 2. Choreic heart disturbance following fright . . . . .152 3. Chorea in a youth following rheumatic endocarditis . . . 152 4. Chorea with hemiplegia. Remarks . . . . . . 153 5. An example of the rapid transitions of chorea . . . .156 6. Violent chorea in an adult. Remarks on treatment . . . 158 7. Chorea of imitation. (Dr. Brabazon.) 163 8. Chorea with muscular paresis and heart murmur in a youth . . 164 9. Chorea of the hand following muscular exertion .... 165 10. Chorea changing from left to right side, joint affection succeeding. 166 11. Hemichorea in a young woman ....... 167 12. Chorea in middle life from mental shock . . . . .168 13. Chorea with general muscular paresis. Benefit of mechanical support 169 14. Chronic chorea with incontinence of urine, &c. Improvement under discipline .......... 171 15. Chronic chorea in a boy of eleven ultimately fatal .... 172 APPENDIX B. TABLE A. 132 consecutive cases of chorea, showing its rheumatic connec- tion, state of heart, exciting causes, limbs affected, &c. . 175 TABLE B. 45 consecutive cases illustrating the same points as Table A, but IN older subjects 187 INDEX 195 Erratum. Page 147, line i,for hopeless ;'^ai/ helpless INTRODUCTION. THE PLACE IN NATURE OF FUNCTIONAL NERVOUS DISEASE. There exists in some quarters a disposition to discredit — and even to deride — the reference of disease, and especially of nervous disease, to functional causes. * Functional or unsubstantial diseases,' it has been said, * are flitting before the microscope like ghosts at sunrise. Nervous disorders, the most evasive of all, are becoming tangible.'^ I am not about to dispute this assertion, but rather to ask what it means. Functional disorder of some degree, I suppose, everyone must admit, inasmuch as the elements of such disorder are to be met with universally. Granting that there is a wide difference between ordinary emotional spasm and hysteric convulsion ; or between the temporary fidgetiness of shyness and the long-enduring agitation of chorea, yet still there is a region intervening which is occupied by muscular disorder of an equivocal kind. The question is, what degree of such disorder is to justify the anatomist in making search for its material expression? No one will answer any degree of it whatever, since that would be to include the whole of mankind, and would involve the absurdity of making disease a universal possession. We are entitled to ask, therefore, how far and in what manner that orderly action of the voluntary muscles which hypothetically represents health may be departed from before disease becomes ' tangible ; ' if indeed we may not ask * The Art and Science of Medicine. Introductory Address at St. George's Hospital, by Dr. Dickinson. B 2 CHOREA. yet further upon what authority any particular method of muscular conduct is insisted on rather than another as being its proper pattern, all declensions from which are to be regarded as morbid. No one will deny, I suppose, that the several conditions of consciousness find a ready and accurate expression in the move- ments and attitudes of the body. So peremptory, indeed, is this union between the mind and the muscles that the term * voluntary ' commonly given to the latter is only partially applicable. It is true that the more violent gestures get subdued and toned down by education, and that custom and imitation supply a certain number of conventional and meaningless movements, yet the muscles are not the less expressive on that account. We are seldom deceived as to the nature of emotion on account of the effort of repression which is used. On the contrary, we can see and measure the force of both. The monotony and uniformity of civilised life tend of course to reduce these motor phenomena to a minimum. The full display of passion or emotion is rare, and when it occurs the sympathy of the observer is so far enlisted as to divert attention from the mere muscular condition apart from what it expresses. Thus the several modes of response to as many different states of consciousness, the spasm of horror, the restlessness of embarrass- ment, the tremor of fear and sudden disaster, violent and dis- composing as they are physically considered, pass without notice, or are noticed only for the meaning they convey, just as the movements of the tongue and lips are not considered in themselves or apart from the words they utter. What would be really surprising would be the absence of these several emotion movements, or still more, the expression of any one emotion by means of muscular move- ments such as we are accustomed to associate with another. Thus while many phenomena of movement which are involuntary and disabling are readily accepted as amongst the natural endow- ments of the individual no less than are the acts which arc INTRODUCTION, 3 voluntary and deliberate, yet must it be provided that each of these shall have its proper motive. Anger excites spasm, fear tremor, mental embarrassment restlessness, and so on. A tremor with no particle of fear or dismay to justify it, a rigid spasm with no sensation of anger or suspense, a restlessness of the limbs with the mind at rest — these are conditions which require a separate explanation. The ordinary laws of emotion movement do not provide for them. Some extra and separate motive must be sought for. But while thus ascribing to emotion a great variety of mus- cular phenomena, it is necessary to remember that this particular motive extends over a wide range including even in some individuals the ordinary conditions of existence. In some temperaments and at some periods of life the most common incidents will suffice to provoke emotion. Familiar illustration of this is seen in child- hood, whose ordinary movements indicate a ready and constant yielding of this kind. Again, the spasmodic movements and involuntary gestures of youth are due to such nervous exaltation as is with them habitual, and, indeed, proper to that stage of physical development. And we may say generally that what oc- casions emotion in one, with its accompanying bodily disablement, will brace and nerve another to his fullest capacity. We cannot say beforehand how or when emotion phenomena will be excited. We know only that, while different individuals are variously re- moved from emotion, there is no one altogether beyond its reach; and that the response to such emotion is by means of particular movements, spasms, and palsies common to all mankind. But we need not confine ourselves to emotion alone, we may consider as well the ordinary movements of intelligence, those muscular acts by means of which we are always expressing during our waking hours the common current and transitions of thought and sentiment. Such movements, although of the same insti- gation as the emotional, are so directed and governed by the will as to subserve the purposes of intelligence. The period of life B 2 4 CHOREA, when these movements are the most precise and orderly is that of early middle age, when the muscular frame, while it maintains stillness without obvious restraint, exhibits at the same time by the disposition of the limbs and features the ability to move with alertness, and to use the muscles for intelligent expression without surrendering them to spasm or palsy. He is manifestly the best upon whom this self-imposed control sits the easiest. Rare any- where, such composure is hardly to be looked for until such time as the comparative calm of middle life has been reached. And what always finds place in such a deportment, side by side with the various sentiments that are animating it, is the continual, watchful presence of self-control, a control of which the highest exercise is seen in the power to maintain a vigorous, capable, un- constrained stillness of the whole frame. This rare perfection of muscular government, I say, is the end and outcome of a lengthened training having for its object not so much to direct the movements as to restrain them. The limbs which are thus disposed have but lately emerged from a condition of continuous, impotent movement where they have been swayed about under an influence lower than the reason yet overpowering it. Even now, if anything should occur to disturb the even balance of the mind the restless, motiveless movement of the earlier time would reappear.^ The best and fullest power of control, indeed, never becomes altogether spontaneous and self-acting. It is not only easily overborne by passion and ^ It will be seen in the sequel that familiar and widely recognised facts such as these are expressed with great variety of phrase. Thus, for example, Professor Bain speaks of a 'central spontaneous energy or activity as a fundamental and per- manent property of the system.' ' The active display of the muscles,' he says, ' is most usually and abundantly brought into play by the stimulus of our various feelings, yet is there no reason for supposing that dead stillness would be main- tained if outward prompting were withheld.' He further speaks of the restless activity of childhood as ' only to be accounted for by a central fire that needs no stirring from without.' — See Bain on The Emotions and Will, Part. II. Chap, I. What I desire of the reader throughout this discussion is that he will separate facts from expressions, and not accept any technical paraphrase or generalised statement of phenomena in the light of new knowledge. INTRODUCTION, 5 emotion, but its constant exercise is so difficult and irksome that any occasion for being rid of it is readily taken advantage of Herein we have one of the uses of laughter, that half-voluntary spasm which in many states of mind affords welcome relief from complete muscular order. Again, the best deportment will seek relaxation by means of a variety of distortions, stretchings and grimaces so soon as these may be indulged in without obser- vation. Such acts are proportioned, as everyone knows, not to the mere bodily fatigue, but rather to the degree of constraint that has been called for, extreme ceremonial or extravagant gravity needing a larger measure of such relief than the common busi- ness of life. It is easy to see that the circumstances may be such as to make the natural craving for this kind of indulgence quite irresistible, insomuch that it has to be yielded to in public as well as in private, and gets noticed and stigmatised as disease. And along with this careful control of movement in the presence of others and its relaxation afterwards, we have to con- sider the obedience that we owe to a conventional code of conduct. Although, as we have seen, there can be no actual uniformity of muscular response, but each of us must act after his own prompt- ing, yet is there a strict pattern set up for general imitation. Whoever errs from that pattern, as by too ready or too violent a display of movement or of spasm, soon finds that these extra acts of his, which would be accepted and allowed if appearing upon proper occasion, subject him to express notice, and (during the tutored period of life at least) to rebuke or ridicule ; and, inasmuch as muscular obedience is more difficult when it is thus observed upon and mistrusted, and the loss of confidence in movement is an extra incentive to error in movement, we have here conditions which must tell directly on the side of disorder. It thus appears that muscular response varies very widely with the individual, the time of life, and the circumstances. We can give no stricter account of it than this : that it will be such or such, according as the conditions in their different associations combine 6 CHOREA. to make it. With certain methods of movement and of spasm, as- cribed generally to emotion, we cannot say beforehand what is the material which each individual will find sufficient for emotion, the very same cause which disables one bracing up another to his best response. With certain conceptions regarding the voluntary muscles as obedient agents of the will, and moving only at its bidding, we find, in fact, that their natural and untrained state is one of con- stant movement, which is so imperative that the best discipline, far from controlling it absolutely, serves rather to disguise its necessity by giving it some show of purpose. With a certain ideal pattern as to the demeanour which is the fittest, we never actually attain such demeanour ; it is not even approached except by long and careful training, and the effort that holds it is always ready to escape. With certain aids to orderly muscular conduct provided by the public sentiment, we perceive that, after a while, the pre- servation of a set attitude becomes intolerable, and that the help which outside observation gives at first to order and stillness, it gives at last to disorder and involuntary movement. I am not asking now how much, or how little, of muscular disorder actually arises from this wide reach of emotion, or this infirmity of control over movement, or this disturbing influence of having to conform to a pattern ; I only say that we have here the elements of dis- order — material out of which it is inevitable that disorder should spring and grow. Yet to what degree such disorder may spread under the most favouring conditions, or what particular pheno- mena of so-called disease may be thus accounted for, is a separate question. And while we thus perceive that the muscular control is variously evaded at the successive periods of life, and take it for a law that the novelty of childhood, the access of new passions in youth, and the monotony of old age should each in turn serve to produce the characteristic movements and attitudes of those periods, it is easy to see besides, how, in a community like ours, the several causes of muscular disobedience and disorder should INTRODUCTION, 7 press unequally at different ages. It is to be observed, especially, how the motor infirmities of early life get confirmed and intensi- fied by the treatment they meet with. Thus, for example, the natural restlessness of childhood is directly fostered in having to bear the additional strain of observation and rebuke. The pre- vailing methods of education, which are designed to repress movement, serve, in fact, only to disfigure it. The attempt to enforce stillness of the body at the same time that the mental capacity is being tasked, does direct violence to the natural laws of movement The emotional period of young women is similarly beset by the tax that is put upon it in the readiness of others sometimes to ridicule, and sometimes to pity, emotional displays which might fitly pass without notice. And this extra and artificial strain, which is thus put upon youth in general, might perhaps be still further defined as ope- rating with its utmost force at certain stages of development anci under certain social conditions. There is a time in every child- hood when the desire for movement is the most active and the least within control ; there is also a time when, by our conven- tional rules of education, the penalties that are put upon move- ment are the most severe. The nearest coincidence of these two periods, both of which may be fixed approximately, ought to indi- cate the age when the frequency of movement disorder is the greatest Similarly, at a somewhat later period of life, with the fuller control of movement which age naturally brings, we should expect a comparative calm, until upon reaching puberty a new source of disturbance would have to be encountered. Thus, not only might we predict that the factors of movement disorder, which we see so variously combined, must appear every now and then in such combination as to bring disorder into special promi - nence ; we might go further, and indicate particular periods of life and states of society whose conditions are such as to favour this combination. Such conclusions, as I have said, must be brought to the test 8 CHOREA. of facts. Is the disorder which we thus prefigure actually found; and does it attach by preference to the periods here indicated ? Are its apparent causes of a mental or a material kind ? Does it prevail more or less according to the social condition, or every- where alike ? Are the particular muscles it affects such as emotion selects, or such as structural disease selects ? Is the actual search for its material cause successful or unsuccessful ? The answer to such questions involves the investigation of the several diseases which, rightly or wrongly, have been classed as functional ; and does not concern us now. It is evident, at least, that such an investigation may be undertaken free from any antecedent objec- tion, and that functional disease, although necessarily beyond the field of the microscope, is not ' unsubstantial ' in the sense of being unreal. But the varieties of muscular conduct are not confined to the circumstances of youth, or the special urging of emotion. We have to take into account, besides, such deformities as arise from inherited and acquired tricks and habits, from the natural ten- dency of human beings to copy one another, and from modifica- tions in the use of the muscles which spring from their frequent employment in some particular way. Innumerable movement disorders are of this origin. Take any man or woman out of the crowd, and how many will be found the involuntary acts and spasms which contact with the world has produced or exaggerated; how many movements are purposeless and imitative ; how com- plete is the transition, in gesture, and demeanour, and manner of response, under observation and in solitude — when inspirited and dispirited — anxious or assured. In all these varieties of move- ment response, which is to be regarded as the normal and healthy one ? I do not say, which is the most convenient and useful — for, however precise the definition may be from that point of view, that is mere matter of sentiment and convenience, and has no right to be heard in the question — but which is the proper healthy pattern ? Is it that in which the muscular response is the quickest INTRODUCTION. 9 and readiest, the state of body always verging upon spasm and ready to exhibit emotion by means of the most violent convulsion ; or is it that in which the muscles are under full command and no expression is allowed to escape except such as the will and judgment approve, the rarest and least attainable of all human conditions ? The full significance of such considerations as the foregoing will be best seen by observing how the case stands with the most sensitive muscles of all, namely, those of the face. If only the principle be admitted that there is nothing special in the move- ments of the face muscles excepting in their alacrity of response, and that a mental impression of whatever kind needs only to be deepened a little to make its motor response spread from the features to the limbs, we shall see then, in the study of this particular region, that spasm, and paralysis, and numerous acts of disobedience on the part of the muscles are part of the daily life of everyone ; that, indeed, the precise conduct of the muscles can only be predicted by a knowledge both of the will that informs them and of all the circumstances in which they are placed ; that to speak of the normal condition of the muscular system is to speak without precise meaning. Any example taken from some more restricted field, with a view to illustrate this position, might no doubt be so used as to misrepresent it. Yet, keeping within the strict terms of the comparison, we may say of the muscular system, as of any system of mechanism, that so long as it remains competent its conduct will vary with its adjustment, and that such variation is the test of its integrity. The most approved clock for present service is the one whose minute-hand occupies precisely an hour in making the circuit of the dial ; but the best clock is the one that is most obedient to its regulator, moving quicker or slower accordingly. But, while we are thus compelled to admit motor disorder as a necessary ingredient of life, we do not admit it indefinitely. There are the muscular phenomena of functional origin — spasm K) CHOREA. or tremor, or palsy — and there are the very same conditions due to substantive disease ; nor would it be difficult to point out, in general terms, the different associations of the two. I am con- cerned at present, however, only to show that there is a place in nature for functional nervous disease ; my next object will be to make out the claim of a particular affection to occupy that place. There is in the mind of each of us a certain pattern of muscular capacity and muscular control, as it ought to be, and there is the larger and less definite pattern which comprehends the actual range of muscular conduct under all circumstances. It is because these patterns are not co-extensive that we need the service of such a word as functional disease to express the difference between them, and occupy the ground which the narrower of the two leaves uncovered. TI CHAPTER I. GENERAL DESCRIPTION OF CHOREA. Pedigree — Definition — Parts affected — Degrees of severity — Mental involvement — Temperament — Age — Sex — Adult and senile chorea — Geographical distribu- tion — Causes — Varieties — Progress — Paralytic symptoms— Recurrence — Con- nection with heart disorder — ^Sequelse. In attempting to give some account of the history and relations of chorea, I deem it unnecessary to describe the various dancing epidemics from which it takes its name. Chorea for us begins with its definition by Sydenham. It may, indeed, at first sight seem strange that a movement extravagance of the middle ages, having its origin in pious fervour, finding its subjects amongst the emotional and the credulous, and its cure in some supernatural agency, should have transmitted its name to a purely muscular commotion of children to which the patients are often themselves indifferent, while they are but ill-furnished with that religious sentiment which provided both the cause and the cure of the ancient disorder. It is remarkable that every epidemic of the kind of which we have any account grew and spread out of material of its own pro- viding. It was never that with the decline of the disorder the terror subsided ; it was always the converse of this. The epidemic disappeared as soon as it ceased to inspire the terror or attract the attention, which was one of its essential factors. In other words, the gesticulations and dancing (although in great measure involuntary) had the strength taken out of them when the popular tribute to their reality was withdrawn. So long as the priests' 12 CHOREA. exorcism or pilgrimage to shrines was held to be necessary, so long, to the great annoyance of their victims, the muscular con- tortions flourished and spread ; but they could not survive neglect It will be seen from Hecker's account of these popular seizures that they did not decline from want of subjects, but from a change in the public sentiment The patients who came latest were discredited. They were not impostors any more than the others, except so far as the knowledge that they would be so ac- counted tended to make them so. The same affection, that is to say, neither more nor less real than at first, had lost the necessary element of public sympathy. The current of opinion changes, but men and women remain the same. There is ample evidence in the experience of the pre- sent as well as in the dancing and jumping manias of the past, that conscious convulsive seizures need external support for their perfect exhibition. Wanting this, the explosive violence of their discharge (to adopt the language of current pathology) is di- minished. Thus, while the example of others suffering, and the knowledge that bystanders are disturbed and impressed, combine to make the circumstances most favourable for the development of such epidemics, the knowledge that the particular symptoms will be recognised only to be discredited, and that those who yield to them are neglected or despised, make the circumstances most favourable for their suppression.^ VVe are not too readily to assume that the chorea we are now to consider is altogether different from the epidemics of old. 1 Upon this subject, in reference not only to popular movement distortions, but also to witchcraft and modern miracles, I would refer the reader to Lecky's His- tory of Rationalism in Europe (vol, i. p. 89, et scq.), where it is well shown that the belief in all such phenomena, genuine alike on the part of performer and ob- server, required for their due exhibition the joint operations of the two. They resulted from ' a general predisposition to see Satanic agency in life which grew from and reflected the prevailing modes of religious thought, and declined only when those modes were weakened or destroyed." With all the distinctive features of our present chorea, we shall find in the sequel, if I am not mistaken, that this affection in many of its modes is largely influenced from without. GENERAL DESCRIPTION. 13 The very same form of muscular derangement that Hecker de- scribes is with us still, in such dwarfed dimensions as the altered circumstances permit. Its motives and methods of cure are the same now as formerly, and if it is less common or less contagious, the reason is to be found in the wider spread of education. But the healthier public sentiment which has overwhelmed in neglect and contempt, together with witchcraft and sorcery, the various movement extravagances due to ecstacy and fanaticism, has brought into greater prominence the muscular disorder of child- hood which we now call chorea. The first mention of chorea indeed as a disease peculiar to children coincides with the time when the growing influence of education had acquired strength enough to deprive the popular epidemics of their neces- sary motive. Along with this change the disorder ceases to be regarded from the old point of view, and descends to a different class of subjects. The notion of demoniacal possession is abandoned, and attention is rather directed to such features of the malady as tend to link it with ordinary disease. It is due to a materies morbi in the blood, to the lodgment of fibrinous clots in the brain, to a new development of articular rheumatism. Such altered attitude of the observer makes an alteration as well in the thing observed. Chorea has changed its shape for reasons which we can partly explain, yet some of the old hneaments are still discernible. The motor disorder which now plagues our children still clings to the same sex as of old, it chooses by preference the same temperament, and is apt to be aroused by precisely the same causes. Of the many attempts that have been made in our own day to define chorea^ none can be regarded as altogether successful It has been described as 'an irregular convulsive action of the voluntary muscles,' but it is not necessarily convulsive, and not always confined to the voluntary muscles ; as ' a tremulous, ir- regular, and ludicrous movement,' but there is nothing of tremor 14 CHOREA, about it, and not much that is ludicrous. Sydenham's com- parison of chorea to the feats of Merry Andrew is familiar to everyone. The more modern definitions are confessedly faulty and inadequate. In the latest treatise upon the subject Pro- fessor Ziemssen declares that it is only possible as yet ' to aim at a definition.' In his own description he is chiefly careful to dis- criminate between true chorea and the so-called chorea major ; the latter as he believes being 'the product of hysteria or of genuine psychoses and cerebral maladies.' ^ By true chorea he understands ' a neurosis of which the seat (as it seems) may some^ times be the brain alone, sometimes the entire nervous system ; characterised by incessant twitchings or jerks of groups of muscles, which are sometimes spontaneous and sometimes excited by voluntary impulse, which occur almost exclusively in the waking state, and are accompanied by a more or less developed psychi- cal disturbance.' It may be questioned whether such definition really pictures the affection to anyone not already familiar with it. To ordinary observation, at all events, chorea consists in an exaggerated fidgetiness. It is an extravagant exaltation of that continual unrest which is the natural characteristic of child- hood. Its movements, that is to say, resemble the emotional, the same muscles being affected in the same kind of way. Chorea, therefore, may be imitated as emotion may. But up to a certain point only; extreme chorea, like extreme emotion, is beyond imi- tation, for there belongs to both a degree of spasm and dis- tortion which can never be assumed, or only partially and for a moment. Consistently with this comparison the muscles of the vpper part of thi body are much more often affected than the rest, and the hands suffer most of all. The thoracic muscles seldom escape altogether, but it is only exceptionally that their concern in the disorder is a prominent feature. Tlio lower limbs arc never ' Zicinsscn's Cycl., vol. xvi. p. 418. GENERAL DESCRIPTION. 15 affected alone, although they may be the first attacked. * In the chorea of elder children and of grown-up girls the face is almost always concerned. The more disturbed limbs, or those in which disturbance lasts the longest, become eventually weakened, the loss of power being sometimes very obvious. Certain groups of muscles indeed may appear to be altogether disabled. Thus the hand may hang from the wrist, and even for a while be incapable of full extension, or, less commonly, the arm or the leg muscles may become relaxed and motionless. Complete paralysis, how- ever, is rare, and, as a very general rule, there is neither failure of nutrition, loss of tactile sensibility, nor alteration in the muscular response to the induced or continuous current Chorea is commonly more marked on one side than on the other, and not seldom it affects one hand, or one anii, in especial.^ For reasons that will presently appear, much stress has been laid upon chorea confined to the arm and leg of one side, so-called hemicJiorea. M. See, in 154 cases, found chorea confined to the left side, or 7nore marked there, in 97. But this is obviously not to the point, since it fails to distinguish hemichorea in any accu- rate sense. Dr. Pye Smith, however, found 33 out of 150 confined to one side. For myself, I cannot recall a single case of chorea of any severity continuing throughout its course limited to one side of the body and including both arm and leg. That chorea commonly begins in one side, we all admit ; that it may continue confined to a single limb until it gradually disappears, we may admit also ; but a violent chorea of one side, in which the other side in no degree shares, is, I believe, almost unknown. The speech defects of chorea are of many kinds and degrees. Sometimes speech will be rendered difficult and uncertain owing to imperfect command of the respiration, causing occasional ^ So Romberg, Sydenham Society's Trans., p. 56, with which my own obser- vation accords. 2 Compare with the statements here given the figures quoted in Appendix B, in reference to the starting-place of chorea. i6 CHOREA. arrests in speech, or the words to be violently jerked out.* At other times there is difficulty in setting the vocal apparatus going ; the answer to a question will be delayed, as though the other muscles were so busy as to leave no place for speech. In extreme cases of this kind, the lips will be seen to move, but the attempt to utter word or sound will go no further. Another kind of speech disorder arises from imperfect muscular co-ordination, and is seen best in the elder patients, giving to their words that indistinctness and confusion of syllables which is characteristic of drunkards. These speech disorders are in no constant relation to the choreic disturbance elsewhere, and, with severe chorea, speaking may be little or nothing affected. But where speech difficulty occurs, it is commonly an early symptom, and may be the very first. That it may be rightly appreciated, it is well to bear in mind that speech chorea, like chorea in other parts, is a muscular disorder. Aphasia, or any other form of misuse or disuse of speech due to cerebral defect, and not to faulty working of the apparatus of speech, is not met with — or, certainly, is very rarely met with ^ — in chorea. Defects of deglutition^ less common than those of speech, follow much the same rule. It is not that the power of deglutition fails (although even this may occur), but that, in severe cases, there is first the difficulty of food reaching the mouth, and next the diffi- culty of its being dealt with by the tongue and lips. The body temperature is almost always slightly raised at first, in the more violent attacks ; but, with remarkable uniformity, // tends to fall below the normal afterwards. It will be easily under- stood that in the utmost severity of the choreic paroxysms trust- worthy observation upon this point is impossible. The highest temperature I have succeeded in taking was ioo-6 ; the lowest, ' Ziemssen, loc. cit. p. 436, has called particular attention to the various laryn- geal defects of chorea. My friend and colleague, Dr. de Havilland Hall, has made many attempts to inspect the larynx in cases of children with marked speech chorea ; but for obvious reasons this is a task of much difficulty. '■* See case 14, Appendix A. GENERAL DESCRIPTION. 17 95 "8. Contrary to the opinion of Ziemssen {loc. at p. 440), that the body temperature is not changed even in severe cases, I be- lieve it to be very rare for the normal heat to be maintained, although the variations are not considerable. The pulse is com- monly quickened, it is often variable, and sometimes uneven or distinctly irregular. During severe paroxysms it may exceed 120. The state of the circulation, however, will come for separate con- sideration presently. The degree of severity of chorea may be well measured by ob- serving the patient's posture in bed. One effect of the continual working of the arms and shoulders is to push the body low down in the bed, thus drawing the head from the pillow, and bringing the whole frame into a horizontal plane. At the same time, the patient remains constantly on the back, so as to allow full play for the extravagant limb movements. Lying thus, the reckless disposition of the arms in their short intervals of rest is charac- teristic. They remain, so to speak, where they fall ; the flexed arm and wrist lying over the chest, or the limb hanging down loosely over the bed-side. With its prone position and the depression of the head and shoulders chorea thus comes, by an accident, to resemble enteric or typhus fever. . In the worst cases, indeed, when the violent move- ment has given place to prostration, this resemblance of posture is complete. It may be added that in severe chorea, as in fever, the position in bed from day to day — a little higher, or a little lower — is a valuable indication of the progress to better or worse. In the commonest form of chorea — that, namely, which is found amongst young children, and is of long duration but moderate degree — two varieties are to be recognised. In the one, the patient's movements are at their worst when he is conscious of observation, and at their best when he is left to himself In the other, there is a considerable power of control when the effort is made ; but without it, as when at play, or in simple muscular acts, the unsteadiness and disorder become very c 1 8 CHOREA. apparent. This latter variety is by far the more untractable of the two. Yet, even in severe chorea, it is less true to say that the child cannot be still than that it is unable to maintain stillness. By an effort and for a moment movement can be stopped wholly or partially. And it is curious to notice the effect produced by making appeal to the child's understanding. If, for instance, he is invited not to be still, but to move to the utmost, it will com- monly happen that the character of the movement is for the time altered, and probably diminished. Similarly, attention directed to a particular limb serves, in a measure, to quiet that particular part. Notwithstanding the inconvenience of chorea, young children take it very composedly. They eat and sleep well, and are in no way mentally disturbed : the mental disturbance comes later. Thus, it is common to find after the age of ten that at the first onset of the affection, or even before it, the child becomes fretful, or capricious, or passionate. It is easy to understand that with these elder children the temper is continually tried by the disobe- dience and riot of the voluntary muscles. At a later period still — at and after puberty — the ceaseless agitation of the limbs, in spite of the will, produces a state of mind little short of terror. It is at this time of life exclusively, so far as I know, that chorea becomes blended, in various degree, with delirium and acute mania. The utmost mental disturbance of the children concerns the temper and the degree of intelligence. It may be true, perhaps, that chorea tends in a measure to blunt the mental acuteness ; but in judging of this we are to remember that the instruments of intelligence are themselves impaired, that failure in act tends to produce infirmity and distrust of will, and that the facial deformity of chorea often so disguises the natural expression that it is no longer a fair index of the mind. Added to all this, the motor disorder so far occupies the attention that it is difficult and irksome to direct it elsewhere. GENERAL DESCRIPTION. 19 Some authors have laid much stress upon the independent variation of two separate elements of chorea, namely, the muscular restlessness and the ataxy. It is pointed out that children with but little restlessness may yet show marked want of muscular consensus, and the converse. It will suffice, in this place, to admit that the fact is so ; its explanation must be sought when we come to consider the pathology of the affection. As regards the kind of person most liable to chorea, indepen- dently of the question of age, or sex, or of predisposing diseases, it is enough to say that of those who have wTitten with authority upon the subject some have chosen the dark children, and some the light. All have agreed, however, that susceptible and ' nervous ' individuals are especially liable, although none believe that chorea is limited to these. ^ The attempt to connect anaemia with chorea I believe to depend on erroneous or too limited observation.^ The favourite age of chorea is between six and fourteen or fifteen. Its period of dangerous and sometimes fatal severity is from thirteen to about twenty-three. Of See's 191 cases, 151 fell between six and fifteen years of age ; only 1 1 w^ere under six, and 12 over twenty-one. In Dr. Pye Smith's^ 136 cases, 106 were between six and fifteen. In 71 cases of Dr. Dickinson's, treated at the Hospital for Sick Children, 42 were under ten years.* In 177 consecutive cases under my own care, 96 were under that age. It is thus necessary to amend Sydenham's statement, giving the limits of chorea between ten years old and puberty. It is rather from the period of second dentition to about twelve or thirteen that it prevails, and then suddenly declines. Yet it must be added that the most formidable examples of the affection occur at the ^ See Appendix B, in reference to the large proportion of whooping cough in choreic subjects. ^ It has been obser\-ed that choreic children in larger proportion than others have large pupils, but I know of no reason for this, and have no accurate data con- cerning it. ' See Guy's Hospital Reports, 1873. * Med.-Chir. Trans., vol. iix. p. 30. C 2 20 CHOREA. time of puberty. If, indeed, such cases were eliminated, the actual mortality of the disease, small as it is, would be very greatly reduced. Chorea occurs to girls more than twice, but so??iethi?ig less than three times, as often as to boys} This sex difference is the least among the little children and the greatest amongst the elder ones, but it never disappears. The statement that there is no sex dis- parity under nine years of age is certainly an error. The statistics of many authors upon this point are in substantial agreement. M. See, in 531 cases, had 393 females, and 138 males; a pro- portion of 2*8 to I. Dr. Hughes has 73 females to 27 males ; a proportion of 2*5 to i. Dr. Dickinson, 50 to 21 ; a proportion of nearly 2-4 to i. Dr. Peacock, 66 to 26 ; or 2-6 to i. My own tables give 99 to 33 — exactly 3 to i — for the Children's Hospital ; and 35 to 10, or 3*5 to i, for the Westminster Hospital. That there is a somewhat less inequality in the younger children — yet no approach to equality — appears probable from the fact that of 21 children under eight years, of Dr. Dickinson's table, 13 were girls and 8 boys ; while of 40 children under the same age of my own, 28 were girls and 12 boys. Of the exact proportion of the sexes at the age of puberty, I cannot speak ; but there is undoubtedly a very large preponderance of young women. A severe form of chorea at the approach or completion of puberty in girls, is a not very uncommon occurrence ; while the same thing in youths is certainly very rare. Cases have been reported of chorea occurring to infants at the breast, and even at the hour of birth ; but I know of none such. Wc have yet to notice a form of the disorder, which, although slower, less varied, and more nearly rhythmical than ordinary chorea, may properly be called by that name. In the very rare occurrence of chorea for the first time in old age, the patients are usually weakened in intellect or actually demented. Less rare, but still uncommon, is . the persistence into adult life of a chorea * See Appendix B. GENERAL DESCRIPTION: 2i that began in childhood ; here, too, there is commonly some degree of mental impairment. Both these forms of the disorder may be regarded as incurable. Professor Charcot, who has called especial attention to senile chorea, has no anatomical pathology for it. He regards it as a merely emotional disease, and quotes examples from the Salpetriere Asylum to show its origin in dis- tressing events and violent passions, as well as the mental feeble- ness that goes along "with it.^ Along with this adult chorea, mention may here be made of the various muscular tricks and twitches acquired early in life, and which, as the common observation of everyone will admit, cease to be curable when youth has passed. There is hardly an individual of middle life to be met with who has not some habit of the kind, which only needs to be exaggerated or extended to be accounted chorea. These movement infirmities concern chiefly the face, head, and shoulders ; and the jerking or twitching of these parts is often strictly rhythmical. ^ The equality of the intervals is seen best when the individual is walking, by their leaving time always for the same number of steps. Such disorder is of course more or less noticeable according to its seat and extent, and is more readily accepted and overlooked in the old than in the young. There is another form of rhythmic movement which is intimately associated with hysteria and with young women, to which M. Charcot has given the name of 'rhythmic chorea.' In this affection, the arm and leg of one side may be alternately flexed and straightened ^ for a considerable period, or the head may be set shaking, and continue to move with constantly in- creasing rapidity, to the utter exhaustion of the patient. These ^ ' On Chorea in Old People.' Lectxire by Professor Charcot, Medical Times and Gazette, March 9, 1878. See also, in reference to post-mortem appearances, ' Cases of Choreic Convulsions in Advanced Age, ' by Dr. Macleod. — Jourit. MeJital Science, July 1881. In Appendix A, case 12, is an example of chorea occurring to a man in middle hfe, intelligent and unemotional, and with no history of any previous attack. 2 In reference to the natural tendency of over-movement in adults to become rhythmical, see Medical Times and Gazette, Septembe 28, 1878. 3 British Medical Journal, vol. i. p. 224. 22 CHOREA. latter examples of perverted movenient are intimately connected with perverted mind, and belong properly to the subject of hysteria. As regards the geographical distribution of chorea., although we have no statistics to offer, it is known that the prevalence of the disorder varies very greatly in different parts of the world. Over the continent of Europe, or, so far at least as medical observa- tion extends, chorea is nowhere very uncommon. It is a familiar disorder, at all events, in France, Germany, Austria, Italy, and European Russia, as well as in the United States of America. But there are other quarters of the globe where it is believed to be far less common. In Northern India I am informed it is very rarely met with, and in Bombay, I learn from a Parsee gentlem.an, a student of the Grant Medical College, that in the Jamsetjee Hospital for natives, where there are 500 beds, he saw, during three years' attendance, only a single case. Dr. Hughlings Jack- son mentions, on the authority of an African medical man, that the affection is very rare among negroes. Dr. Livingstone makes no mention of chorea amongst the diseases he encountered during his travels. It is curious to notice that in countries where extra- vagant movements and contortions are practised in connection with religious rites, this movement disturbance of childhood, which takes its popular name from a mediceval superstition similarly allied to religion, should be rare.' There is no evidence to show that chorea as such is heredi- tary ; yet, inasmuch as the temperament which exhibits it most is often inherited, it is probable that in certain families the affection should appear with unusual frequency in successive generations. To sum up, then, the ascertained facts in regard to chorea ; we find its favourite time of life included between the time of * How far chorea may be influenced by social position is a question of much interest, but also of much difficulty. I Ijclieve the fact to be that chorea as z. fully developed and recognised disease is very much commoner with the poor, while among the well-to-do we have as many or more examples of commencing chorea, which due care and timely relief succeed in arresting at that stage. We must speak of this presently. GENERAL DESCRIPTION. 23 second dentition and that of puberty ; its favourite sex is the female ; the temperament that invites it most is the sensitive and emotional ; it is apt to increase in severity as it decreases in frequency, little children suffering quite commonly, yet hardly ever with a fatal result, whilst of the few instances of adult chorea a notable proportion have died. Further, the disorder is but little if at all hereditary, and more common in highly civilised countries than in other parts of the world. So much is certain ; what is to follow is to some extent involved in controversy. The exciting causes of chorea, its clinical and pathological associations, com- mon sequelae and modes of progress or of cure, furnish so much material out of which every student of the disorder would obtain a sanction for his own conceptions as to its real nature. It is impossible to proceed with the further study of the subject free from the bias of some hypothesis or other which will interpret and select facts which are equivocal in its own interest. I shall endea- vour in this place to give the commonly received opinions upon the points just enumerated, and with this to conclude a general account of the clinical aspect of the affection which shall be free as far as possible from matters of speculation and difference. Be the exciting cause of chorea what it may, there is cfm- monly a distinct i7iterval hetween cause and effect. It s pro- bable that such interval is really shorter than it appears, inasmuch as the disorder is seldom observed at its very commencement. Dr. Hughes ^ made special inquiry into this point in 26 cases. He found that in 13, or exactly half of these, this interval was o not more than a week, in 2 of the 26 it was between one and two weeks, in 3 it exceeded two weeks, and in 8 there was no ap- preciable interval. Thus in all but five the attack followed its presumed cause either immediately or within a week, the latter ■ being the commoner event. The ijiimediate exciting causes of chorea are variously stated by different authors. It is certain and universally admitted that ^ Guy's Hospital Repoj-ts, 1846, p. 380. 24 CHOREA. fright and mental disturbance are prominent amongst these causes. Certain also that various kinds of pain, rheumatic and other, headache, joint pain'^, and the like, precede and accompany the affection whether as cause or not. Again, the convalescence of children from acute disease, scarlatina, measles, rheumatism, is some- times disturbed by the appearance of chorea. This liability seems to attend especially the recovery from acute rheumatism. But in what proportion of cases this occurs, and whether or not this lia- bility holds good apart from heart disease, are matters of contro- versy to be discussed presently along with the whole question of the relationship between chorea and rheumatism. It may be stated, however, as amongst the facts which are beyond dispute, that be- tween chorea and heart disease, whether rheumatic or not, there is during childhood a distinct connection. There remain a not inconsiderable number of cases whose immediate origin is un- certain, where there has been neither rheumatism nor heart affection, nor acute disease. No hypothesis as to the pathology of chorea can be accepted which does not take account of this class. A child is at first supposed to be careless or awkward, and pre- sently is discovered to be choreic. The affection in this kind is often prolonged but seldom violent. We may thus recognise, without however comprehending chorea in its entirety, three degrees of the affection : First, the obstinate, but not severe muscular restlessness of little children, of very obscure origin, gaining little by treatment but recovering at length as it were spontaneously. Secondly, the severe and sometimes fatal chorea of puberty, due often to some obvious mental disturbance, and, in the case of girls at least, never with certainty separable from some such cause. Thirdly, the rare and incurable chorea of the adult, distinct in many respects from the childish affection, being more spasmodic, rhythmical and uniform, and having no con- nection whatever, as I believe, with heart disease. If we add to these a small but distinct class of cases curiously associated with acute rheumatism and endo- and pericarditis, very early assuming GENERAL DESCRIPTION. 25 the form of uncontrollable and often fatal spasm, we shall have embraced the chief varieties of the disease. To these points we shall presently return. ♦ The progress of chorea is very irregular and its duration un- certain. Recovery is commonly gradual and apt to be inter- rupted. In some rare cases it has suddenly disappeared after mental shock. The rule of the affection according to my own observation is this ; that after a longer or shorter duration signs of improvement will commence, but that this amendment is not continuous, but subject to interruption and relapse. Slight trouble or excitement, or physical pain, the sight of other children suffering similarly, or temporary indisposition short of serious illness, are among the common causes of such relapse. But when recovery has reached a certain point it becomes henceforth rapid and continuous. It must be added that when chorea first comes under formal medical treatment it commonly gets worse, while, apart from disturbing causes, rest in bed, especially with the younger children, will commonly make it better. The duration of chorea is too variable to admit of any general statement. Dr. Hillier and Drs. Gray and Tuckwell have fixed ten weeks as the average. It is seldom recovered from under six weeks, and, as has been already mentioned, in the case of very young children, it may linger for many months. It is not easy indeed in any individual instance to assign its precise limits, for it is seldom discovered quite at the first, and, owing to the nature of its subjects, it declines very gradually, not into absolute steadiness, but into such comparative quiet as still leaves the shadow of the old disorder. Honest observers, therefore, of the very same series of cases of chorea, might differ widely in their account of its duration. To these statements regarding the progress and duration of chorea, something must be added in regard to occasional and ex- ceptional symptoms. One curious and rare incident of the affection is the intervention of general or localised muscular weakness. The > 26 CHOREA. chorea, that is to say, will moderate in respect of the violence of the movements, but there will supervene, along with the failure of muscular consensus, a distinct /^55 of power on the pa7't of individual muscles^ the general health meanwhile being maintained, and the bodily weight even increasing. Other things being equal, such chorea is not more dangerous, although it is longer than the ordinary form. In such cases, I think, the decreasing violence of the movement as an augury for good is hardly lessened by the ob- servation of this particular symptom. It is not uncommon for choi-ea to recur, although two, and still more three recurrences are rare. M. See had 37 recurrences among 158 cases. Dr. Dickinson in 71 cases has 18 second attacks, 4 third attacks, and i fourth attack. In my own 177 there are 38 which were second attacks, 10 third attacks, 3 fourth attacks, and I in which the precise number of attacks was uncertain. In the chorea of pregnancy and of puberty it is common to find that the same disturbance has happened in childhood. Thus Dr. Barnes ^ in 66 choreas in pregnancy had 14 of this kind. My belief is that the severe chorea of puberty would also show a large proportion who had suffered earlier attacks, but the numbers at hand are insufficient for the purpose of proving this. Heart affection in cofinection luith chorea will be considered in a future chapter. I would confine myself now to a simple statement of what passes current upon this matter, as well as of what I shall presently attempt to prove. It is universally admitted that a sys- tolic apex murmur is a common symptom in the chorea of child- hood. Ziemssen speaks of this murmur as of purely functional origin, due, as he believes, to the action of the capillary muscles. Upon d priori grounds he considers that change in the valvular sound * is more likely in chorea than in any other affection.' He asserts (I believe with perfect accuracy) that there is commonly * no increase in the second pulmonary sound, no enlargement of the right ventricle, nor any other sign of increased tension demon- * Obstetrical Trans., vol. x. p. 147. GENERAL DESCRIPTION. 27 strable in the system of the pulmonary artery.'^ At the same time he recognises ' the residual consequences of an old endocarditis ' as sometimes contributing to produce murmur. It is occasionally hard to decide, according to the same authority, whether the mur- mur be anatomical or purely functional, and he instances ansemia, chlorosis, and acute rheumatism as exhibiting ' functional disturb- ances of the mitral quite as obscure as does chorea.' In all this I venture to concur, and still further would desire to recognise a ' chorea of the heart,' which Ziemssen describes without adopt- ing the description, 2 consisting of' irregularity of rhythm audible while the disease is at its height, but neither before nor after.' The opinion I have been led to form upon this subject, and which I shall seek to justify presently, is as follows : — Accepting the views just stated as to the frequency and character of cardiac mur- mur and the absence of signs indicating enlargement or change of any kind in the heart cavities, I would insist, too, upon the cardiac symptoms of chorea as being special and unique, a part of the disease itself, coming and going with it, and exhibiting in many instances not only murmur, but marked unevenness of rhythm and even irregularity of action. This cardiac implication is not more frequent in severe than in moderate chorea, and follows no other rule than that it is more common in children than in adults. The irregularity of the heart is in no direct relation to that of the respiration, and seldom gives rise to any subjective symptom. Such description applies only to that functional disturbance of the heart to which all young choreic subjects are liable, although many escape it. We are to remember at the same time that there is at least this connection between chorea and endocarditis, that the disturbance of the heart which begins with an attack of chorea, ^ Loc. cit. p. 440. 2 Ziemssen speaks of arhythmia of the heart as 'certainly very rare,' and quotes Romberg's assertion that he could discover no abnormal variation in the movements of the heart in spite of continued observation. Accelerated action of the heart is the utmost he admits. These points will be found discussed in Chap. III. 28 CHOREA. and exhibits at first no more than uneven or irregular rhythm, may presently develop a mitral murmur, without trace of rheumatism past or present, a murmur which shall . not only present all the physical signs of endocarditis, but which after death shall be found to depend upon fibrinous deposit. It has been mentioned already that the chorea of later life, and especially that of puberty, is commonly associated with more or less of nervous exaltation. In young women hysteria is often com- bined with it. The two affections may occur alternately, or they may be so intimately blended that the one name seems as appro- priate as the other. The same sort of sympathy is further illus- trated by examples of choreic movement in girls being replaced by tremor or insensibility, or even prolonged immobility.^ The loss of will control is further shown in a still rarer modification by Dr. Handfield Jones, who mentions a case where certain words escaped involuntarily, and to the evident mortification of the patient.^ Among the rare associations of chorea is delirium, rising in some instances to such violence as to assume the character of mania. This symptom will sometimes accompany, and sometimes alternate with, or replace chorea, reminding us of a similar conduct on the part of epilepsy. Such nervous implication is confined to the chorea of puberty, pregnancy, or early adult life, thus adding another distinctive feature to the disorder as seen at this period.^ 1 Dr. H. Jones, Functional Nervous Diseases, p. 362, sq. A child (aged six) has lately been under my care recovering from chorea, who exhibited remarkable fixity of attitude, inasmuch as that she would maintain for a considerable time any posture, however inconvenient, in which she was placed. Thus she would remain quite motionless with both arms extended and the head thrown back ; and at ordinary times, although not actually cataleptic, was, to use Dr. Jones' words, 'un- naturally still,' even for an adult. Moreover, when set to walk she seemed to want spontaneity, would soon stop, and only after repeated halts and several biddings get round the table. Other similar examples are quoted in the Medical Times and Gazette, ' Motor Disorders," October 19, 1878. 2 The occurrence of painful points, or points painful upon pressure, ought probably to be mentioned in this place, but I have no observations of my own on the subject. See Ziemssen, loc. cit. p. 435. ' See further references to such cases in Chapter IV, A good example of the kind quoted by Dr. Bradbury, of Cambridge, will be found in the British Medical GENERAL DESCRIPTION. 29 It is not uncommon in the violent chorea of adults to find that whenever by a strong exertion of the will the overmovement is violently resisted, the effect of such effort presently appears in the form of emotional agitation. I have conversed with adult patients, who, after their recovery, still retained a vivid impression of the state of mind which they described as compelling them to surren- der themselves as by a sort of choice to movement rather than emotion. In evidence of the hysteric alliance of chorea, its i77iitatwe ten- dency is sometimes quoted. And that the disorder is sometimes spread in this way there can be no doubt. Thus Bucheteau ^ relates that in the course of five days after the admission into hos- pital of a girl suffering from most intense chorea, eight patients already in the ward contracted the disorder. Dr. West has ob- served instances of a similar contagion. It must be admitted, however, that this mode of conveyance is rare. In many instances, and probably in that just quoted, it is not so much that the disease is directly imitated as that it spreads by the intervention of terror produced by the sight of it. From this aspect of chorea in association with various functional nervous disturbances, we might proceed to consider its connection with structural diseases of the brain and spinal cord. Chorea is sometimes followed by hemiplegia, sometimes it leaves a permanent contraction of the flexor muscles of the wrist, and sometimes such oscillatory movement as is met with in disseminated sclerosis. Again, convulsion or tremor has been seen to alternate with chorea, and epilepsy ^ has both preceded and followed it. Of such phe- Journal, June 10, 1876, the patient being a young man, and the chorea, which was ultimately recovered from, originating in fright. 1 Dr. H. Jones, loc. cit. p. 350. 2 Out of a large number of" cases of epilepsy. Dr. Gowers found but twelve who had had chorea, and in only four of those did the fits begin at the time of the chorea. In eight cases epilepsy existed before the chorea. In the small number of instances where the fits immediately succeeded the chorea, he thinks it probable that ' the impaired nutrition of the motor centres may have left a predisposition to further disturbance.' I think, on the contrary, that the exceptional character of 30 CHOREA. nomena we shall speak more particularly presently. "WTiat I have to insist upon here is that they are all in the highest degree excep- tional We are absolutely forbidden therefore from attributing any such meaning to them as they might justly bear were they of com- mon occurrence in chorea. What is called ' choreic hemiplegia ' is not only very rare, it is seldom complete or enduring. In some alleged instances of the kind, the evidence of chorea is very defec- tive, w^hile in others it is evident that the preceding affection was not chorea but tremor. ^ A form of paralysis far commoner than hemiplegia is that temporary loss of power on the part of a par- ticular group of muscles which has been just noticed. The occurrence of such lesion can never escape notice, for it serves in a marked manner to disfigure the characteristic aspect of chorea. Apart from such exceptional instances, what we have to consider now, — what we see ninety times out of a hundred, is the condition I began by describing, a restlessness of the limbs and faces of chil- the occurrence argues strongly against any such predisposition. See Gower's ' Lectures on Epilepsy,' Lancet, vol. i. 1880, p. 355. ^ In the three cases of choreic hemiplegia related by Dr. Todd [Nervous Diseases, p. 312, et ^eq.) and often quoted, the chorea in th* first (a boy of nine) was not ' hemichorea, ' nor was the paralysis complete ; the patient was nearly well in eight days. The second was a girl of eighteen, who after right-sided chorea appeared uneasy in her right foot, had feeble grasp with that hand, and sensations of numbness in the right arm and shoulder, symptoms not unusual in one of her age and sex. The third case is that of a child of five taken ' with a trembling motion of both arms,' suddenly followed by right hemiplegia, observed only by the mother, lasting only two or three days, and succeeded by right-sided chorea. This last case, therefore, is the only one in point as showing a connection between chorea and hemiplegia of the same side. As contrasting with hemiplegia, some examples of my own may be here shortly quoted, illustrating that temporary or partial loss of power which is not very uncommon in chorea. A boy aged nine, with very chronic chorea, is observed one morning when being washed to have the left shoulder drooping, and is found on ex- amination to have lost power incompletely (and, as it proved, very temporarily) of the muscles that raise the shoulder. A girl aged about ten with severe chorea is unable to extend the right hand when the arm is raised. As an example of more permanent changes, a girl of six gets in addition to her chorea habitual flexure of one arm, with tendency to tremor therein. Another girl, aged ten, after many months of chorea, develops while under observation such contractions of the flexors of the left arm as to keep the hand firmly bent at the wrijt, after the manner of so-called athetosis. GENERAL DESCRIPTION. 31 dren easily borne and not affecting the health ; involuntary more or less, changeful with the emotions, apt to influence the action and rhythm of the heart ; moving the upper limbs in preference to the lower, sometimes limited to a particular set of muscles, but other- wise seldom strictly confined to one side of the body ; liable to recur during the period of childhood and youth, while after that time it is often replaced by hysteria, which mixes with it more and more intimately as puberty is approached ; an affection of very uncertain duration and uneven progress, yet almost always (in the case of children) recovering completely without entailing any further disorder or infirmity. Such, in fact, is a general sketch of chorea, the object of the present chapter. Its exceptional phe- nomena are neither to be overlooked nor overestimated, and will be considered in their place. In what follows we are to examine in detail the various re- lationships and asserted modes of origin of chorea ; to consider the theories that have been propounded to explain it, and to offer for acceptance some such hypothesis in regard to its pathology as an unbiassed review of the facts may seem to justify. CHOREA, CHAPTER 11. THE CONNECTION OF RHEUMATISM WITH CHOREA. Analysis of 177 cases of chorea — Comparison with other statistics — Conclusions in reference to rheumatic connection, (i) from statistics, (2) from general con- siderations. In Appendix B will be found two series of cases of chorea, the one consisting of 132 examples furnished by the Hospital for Sick Children, the other of 45 furnished by the Westmin- ster Hospital. The question of the rheumatic origin and associa- tions of chorea was made a special object of inquiry in the first series, where the cases are taken consecutively, with no sort of selection, and where, moreover, the entries are not mine, but those of successive Medical Registrars at the Hospital for Sick Children during the five years over which the record extends. For these reasons, although the two series tell substantially the same tale, I would direct the reader's attention more especially to the first and larger series. It will be seen, at the place just re- ferred to, that the 132 cases are arranged in three consecutive sets of 51, 49, and 32 respectively; and, although there is no real break between one series and the next, it may give the clearest impression of the question at issue if the facts in regard to rheumatism are stated separately for each of the three. It appears thus, that, of the 51 cases (14 boys and 37 girls), acute rheuma- tism was in the history of only i, and rheumatism in the history of only 6 — doubtfully as regards 3, if not 4, of these ; in 3 the facts could not be ascertained. Of the second series of 49 cases (13 boys and 36 girls), 4 had had acute rheumatism (i of them RHEUMATIC CONNECTION, 33 shortly before the choreic attack) ; 6 had probably had rheuma- tism ; 2 were doubtful ; and in i the facts were not known. Of the third series of 32 cases (6 boys and 26 girls), 2 had had rheumatic fever ; 7 rheumatic pains ; and i was uncertain. Thus, the whole 132 cases give 7 who have had rheumatic fever ; 14 to 1 6 who have had pains probably rheumatic ; 6 who were doubtful, and 5 where the facts were not ascertained. The precise accuracy of this account cannot of course be guaranteed ; but if the facts be stated in somewhat different form, we may assert, precisely and certainly, that of the 5 1 cases of the first series there are 39 with positive evidence against any rheu- matic connection whatever ; of the 49 of the second series, 35 with such evidence ; and of the 32 of the third series, 22 — 97 cases, that is to say out of 132, were free from any rheumatic association. In other words, chorea has nothing to do with rheu- matism in three-fourths of the cases. And when we come to inquire what is the nature of the association, it will appear that of this small proportion of cases having a rheumatic history there are very few where the chorea springs from the rheumatism in such manner as to make the one seem to be the direct consequence of the other. In perhaps 6 out of the 132, or i in 22 — 2 of these 6 being cases of acute rheuma- tism — this direct relationship may appear to exist. Allowing a considerable margin for error, the conclusion to be drawn from these numbers is not doubtful. It would appear from them, so far as children are concerned, that chorea in the great majority of cases, say in three-fourths of them, is apart from rheu- matism altogether ; and further, that chorea, as the direct and im- mediate consequence of rheumatism, is a rare event. It will not be denied that 132 cases, taken consecutively, and where pains were taken in regard to this particular point, afford weighty material to be put in evidence upon the question at issue. But the particular conclusion arrived at may be resisted on several grounds. It may be said that a too rigid definition of rheumatism has been adopted, D 34 CHOREA. which excludes proper examples of it. The symptoms of rheuma- tism, it will perhaps be urged, occur to children with special modifi- cation, and are, on that account, easily overlooked. It may be added that these statistics are in direct conflict with others, the balance of testimony being, on the whole, in favour of an intimate connection between chorea and rheumatism. There is a further contention which will be best considered separately. It is that of some physicians who, while admitting that rheumatism is hardly excessive among choreic children, yet still maintain the rheumatic association in virtue of their kindred. I would say a word or two upon each of these points. As regards the definition of rheumatism^ the account of the patients has usually been accepted. In some few cases there have been joint pains of no specified character, without redness, or swelling, or definite illness. In these, the occurrence of rheuma- tism was held to be doubtful, and the cases are so recorded, and have been included in the above enumeration. As to the peculiar character of childish rheu7?iatis7n, it is true that many authorities lay stress upon the evanescent character of the joint affection in these subjects, and the great frequency with which the heart is attacked.^ Accepting this observation without reserve, it seems obvious to remark that the original assertion of the connection between chorea and rheumatism had reference to the limb pains and joint inflammation of the latter disease as we see it at all ages more or less. If this latent form of rheumatism is now to be substituted and the absence of any history of rheumatism to be explained accordingly, we not only make all former statistics worthless, but virtually admit, by raising this new plea, that the original assertion as to the connection 1 Side by side with the statement that the joint impUcation of rheumatism in childhood may be slight and evanescent, may be put the observations of M. Bouilly upon a febrile condition of children involving the joints called by him ' the fever of gp"owth,' and which, although unconnected with rheumatism, would in all probability be mistaken for it. See Bouilly, Journal Ue Midecine et de Chirurgie, December 1879. RHEUMATIC CONNECTION. 35 between chorea and rheumatism has broken down. And, more- over, if it be by inflammation of the heart and pericardium rather than by joint pains that childish rheumatism shows itself, it will be safest to connect chorea, not with the rheumatic poison (which is a vague thing at best), but with endocarditis and pericarditis.^ It must be remembered, besides, that the more we succeed in making rheumatism a common disease amongst children, the more we must expect to meet with examples of it in any collec- tion of subjects whatever, whether of chorea or any other affection, unless, indeed, which no one pretends, the one disorder excludes the other. The statement that the results just quoted are at variance with general testimony, and that, upon the whole, the rheumatic con- nection of chorea is supported by statistics, is one which need not be accepted immediately. Of continental opinion it may be enough to say that the French and the Germans are very much at issue upon this point. Trousseau, as is well known, always insisted upon the intimate union of the two affections, reckoning, however, as evidence of rheumatism any traces of by-past endocar- ditis. M. See finds chorea and rheumatism coinciding in 6 1 out of 128 cases ; in only 32 of these, however, 01 one-fourth, were the rheumatic signs decisive. M. Roger has disposed of the question summarily, and in language which renders the labour of taking percentages superfluous. He believes that ' chorea and rheuma- tism are one and the same affection under two forms.' On the other hand, Romberg lays no stress upon rheumatism as allied to chorea, and declares his dissent from the views of English writers in this respect ; while Steiner, of Prague, records 252 cases, only 4 of which occurred during the course of acute rheumatism. 1 Senator, in his lengthy monograph upon rheumatism, alludes to the risk of heart disease amongst the young, and adds: 'This' (i.e. the heart disease, and not the rheumatism) ' is not unfrequently followed by chorea, especially when the mitral is the affected valve.' This one sentence contains the only allusion to chorea. So little do the historians of rheumatism respond to those of chorea upon this subject. — Ziemssen, vol. xvi. p. 57. £> 2 36 CHOREA. The most recent statistics of our own upon this subject are those of Dr. Dickinson, in vol. lix. ' Medico-Chirurgical Trans.' ; of Dr. Owen, in the 'St. George's Hospital Reports,' vol. ix. ; and of Dr. Peacock, in the eighth volume of the 'St. Thomas's Re- ports.' There are also the often-quoted cases of Dr. Hughes in the 'Guy's Hospital Reports.' These last, however, owing to their date, and other circumstances which I shall mention presently, seem to require separate consideration. Dr. Dickinson's cases refer to the same hospital, and include the same ages as my own (from two to twelve). In 71 cases, as many as 10 are doubtful (they are 7narked as 9, but a tenth case is described in the same terms) as to their antecedents. There remain 61, in 42 of whom rheumatism is absent ; 19, at the most, had rheumatism some- where in their history, and in 7 of these it immediately preceded the chorea. Two hundred and three cases (these and my own) thus furnish 139 wholly free from rheumatism, 21 without history, and perhaps 43 who had rheumatism somewhere in their lives, in 12 or 13 of whom this affection might be regarded as cause, or part cause, of the chorea. These numbers refer to a child's hospital where the utmost age is twelve. General hospitals, where there is no such age limitation, may be expected to yield a larger proportion of rheu- matism, inasmuch as with the larger aggregate of years there is more time for accumulating disease. In two tables of the model medical reports of Dr. Owen, of St. George's Hospital, will be found a collection of 50 cases, 22 of whom were over twelve years old. Of these 50, 34 were without rheumatism, 3 were too doubtful to be reckoned, and 13 had rheumatism in their histories, in 4 of these last immediately preceding, and pre- sumably causing, the chorea. Adding, therefore, this third list, we get 253 cases, which yield 173 wholly free from rheumatism, 24 doubtful, and 56 who had rheumatism somewhere in their lives, of whom 16 or 17 had the rheumatism in immediate connection with the chorea. RHEUMATIC CONNECTION. 37 In Dr. Peacock's tables, dealing with 92 cases, only a portion of whom were children, there are as many as 24 or 26 who had had rheumatism at some time or other, the rheumatic symptoms having immediately preceded the chorea in 7; 53 or 54 were known not to have had rheumatism ; and the remainder are variously accounted for.^ The percentage of rheumatism, therefore, is higher in these than in the other tables, yet still the number of the rheumatic is only just over a fourth of the whole. There remain for consideration the statistics of Dr. Hughes, in the 'Guy's Hospital Reports ' of 1846 and 1855. It is neces- sary to observe of these tables that they were not constructed exclusively from cases under the author's own observation, and that there is no constant purpose pervading them. In many instances rheumatism is not even alluded to. The first series, indeed, is founded upon the supposition, no longer tenable, that the existence of cardiac murmur deposes directly in favour of past rheumatism. We read, therefore, that while 8 cases only had their origin, 'more or less directly," in rheumatism, 'there were only 15, out of the 104 where inquiries were made on the subject, in which the patients were both free from cardiac murmur and from a previous attack of rheumatism.' Such a statement would not diifer very widely from the general experience. Some sort of cardiac disturbance is very common with the younger children. This observation, however, can no longer be accepted as deter- mining the question of the proportion of rheumatism. In Dr. Hughes' second series of cases, rheumatism finds place in the notes of but 58 out of 209. It so happens that in this casual mention the positive fact of its presence is noted 30 times, and the negative fact of its absence 28 times, or about half and half. But, it is surely unfair to suppose that these numbers at all represent the actual proportion of rheumatism in the whole body * From the elaborate character of Dr. Peacock's report I find it difficult to summarise it, and would refer the reader to the paper itself, which deals with many other points of interest besides the one here discussed. 38 CHOREA. of cases. A small number of examples thus brought together, with a view to illustrate the rheumatic origin of chorea, at a time when such origin is devoutly believed in, will be certain to give undue prominence to that connection. It is only by taking a number of examples consecutively, over a long period, that this disturbing element of choice can be eliminated. Where this is done, the results, as I have shown, are not very various, and non-rheumatic chorea is exhibited in overwhelming excess of the rheumatic, the percentage of the former var>'ing from 75 to 85. But it is said the rheumatism with which chorea is associated appears in the families rather than in the patients themselves. It is impossible, of course, to test this position with any accuracy. Rheumatism is to be found in every family in thp kingdom if we do but seek far enough, nor can anyone say what is the proper normal amount of it. I have noted the point in 73 of my own cases, restricting inquiry to the immediate family, that is, to parents, brothers, and sisters. The result is as follows : Rheuma- tism is in the family history in 19 out of the 73 ; 9 of these 19 have themselves had rheumatism, and 10 have not. In i or perhaps 2 of these 9 personally rheumatic patients rheumatism was probably the direct cause of the chorea, and in i rheumatic fever was the direct cause. I do not quote these figures as con- clusive, or even wholly reliable. So far as they go, and are accepted, they do not confirm the assertion that choreic children have, as a rule, near relatives who are rheumatic. We may well hesitate to believe a doctrine to which the statistics of rheumatism give no support. At any rate, if rheumatic parents transmit their morbid peculiarities to their children, they may be trusted to transmit first of all a liability to rheumatism. We are thus brought back to consider the question as regards the children themselves, since it is certain that if these are choreic in excess, they must still more be rheumatic in excess. There is reason to believe, from the figures that have been quoted, allowing sufficient room for error : — RHEUMATIC CONNECTION. 39 I St. That the proportion of chorea showing antecedent rheu- matism does not exceed 25 per cent, of the whole number where children up to twelve years old are taken (viz. 20 per cent, in my own 132, and at the rate of something under 26 per cent, in Dr. Dickinson's 71) ; and somewhere between 26 and 28 per cent, where there is no age limitation (viz. 26 per cent, in St. George's 50, and 26 or 28 per cent, in Dr. Peacock's 92). 2nd. That the number of instances where rheumatism, in whatever form, is wimediately connected with the chorea is very small ; being 4 per cent, in my own cases, 7 in Dr. Dickinson's, and from 7 to 8 in Dr. Peacock's and in Dr. Owen's (St. George's). 3rd. That in the great majority of cases of chorea occurring in children supposed to have been rheumatic, the immediate exciting cause of the chorea is no other than that which suffices to produce the same affection in other children who have never been rheu- matic. It is obvioue that by no method of argument can these conclu- sions be made to tell in favour of that intimate connection between chorea and rheumatism which some contend for. Let us say, for example, that 100 instances of chorea yield 25 with a history of antecedent rheumatism. Of these there must be a certain number representing the proper proportion of rheumatic subjects, which we might expect to find in 100 individuals. What this number should be we know not. Suppose that, on an average, only 5 per cent, of our countrymen get rheumatism in childhood. We have then 20 out of 100 choreic patients charged with rheumatism in excess of the proper number. But physical pain is a well-recog- nised cause of chorea, so that of the cases that arise immediately out of rheumatism (from 5 to 8 per cent, as we have seen;, some may be accounted for, not because of the rheumatism as such, but because of the pain that attends rheumatism. This would still further reduce the number. The excess of rheumatism, then, in chorea would seem to be represented, at the utmost, by something between 15 and 20 per 40 CHOREA, cent. — an excess too small to be adduced in support of any intvnate alliance between the two, but nevertheless needing to be accounted for somehow. The question, in fact, comes to this : whether to believe that rheumatism confers this extra liability to suffer from chorea, not immediately, but at some future and it may be distant period ; or else to believe that the excess of rheumatism is only apparent, and due to the fact that rheumatism shares with chorea both heart disturbance and limb pains, so that symptoms which properly belong to the one affection are easily attributed to the other. If the first supposition be adopted it will be necessary to insist (for here the evidence of statistics is decisive) — first, that the rheumatism which predisposes to chorea is seldom connected with it in point of time, a clear interval occurring between the disappearance of the cause and the arrival of the effect ; and secondly that the rheumatic children who become choreic require the very same immediate incentive to the disorder as do other children — namely, alarm or mental excite- ment or nervous shock. If the second supposition be adopted, the prevalent belief in the connexion of rheumatism and chorea depends upon faulty observation and is sufficiently explained by the circumstances ; while in the lessening proportion of rheumatism in our later statistics we see the operation of that still recent knowledge which discriminates between the heart affection of chorea and that of bygone rheumatism. But it is not by statistics alone that this question must be settled, nor do the bare figures give a complete representation of the relationship of chorea to acute and chronic rheumatism respectively. With the special liability of choreic children to joint and limb pain as well as to valvular heart murmur, we may well accept a much larger proportion of rheumatism than is actually credited to chorea without becoming convinced of any real connection between them. The prevalence of such a belief is easily accounted for without assuming its truth. The case is somewhat different with acute rheumatism. The instances in RHEUMATIC CONNECTION. 4 which severe and sometimes fatal chorea arises in the course ot this disease are so striking and so similar that notwithstanding the comparative rarity of the conjunction it is difficult to resist the conclusion that the one affection actually gives rise to the other. Such examples, although too few to affect perceptibly statistical tables, are yet numerous in the aggregate. If, indeed, we were to exclude the commonest form of chorea and look only to adult patients the proportion of instances where chorea occurred as the immediate sequel of acute articular rheumatism with endocarditis would not be excessively small. ^ And not only so, but the sequence may be different, and in the course of chorea, or imme- diately on recovery from it an attack of acute rheumatism may supervene. On the other hand, and lest any should think that this connection is other than rare, let it be considered that in a disease so familiar to hospitals as acute rheumatism many physicians of experi- ence have never seen this association, no one would think of antici- pating it, and the voluminous records of rheumatism hardly make mention of it. Further material will be adduced in the course of this treatise, upon which the reader will be invited to form his own , judgment upon this matter ; in the meanwhile, the following conclusions seem to be justified by a full consideration of the facts : — I St. Acute rheumatism with heart implication, although rare in the history of chorea, occurs in such association with it as to justify the assumption of some direct relationship. The combina- tion, however, is so exceptional that it hardly affects the question of the origin of ordinary childish chorea. 2nd. Chronic or subacute rheumatism, although mentioned 1 Mention may be made in this place of 22 cases of chorea recorded by myself many years ago, when Medical Registrar at St. George's Hospital, and not en- tered elsewhere in this book, having as many as three examples of the connection we are discussing. One was a boy of nine, who got chorea when recovering from acute rheumatism with pericarditis ; another was a girl of sixteen, who got acute rheumatism in the course of severe chorea ; the third was a girl of ten, who had acute rheumatism and chorea concurrently. 42 CHOREA. in the histories ot patients more often than the other, is so difficult of identification, so often disconnected as regards time, so seldom seen unequivocally in actual company with chorea, and so easily imputed upon insufficient grounds, that the asserted influence of the rheumatic diathesis, or indeed any real connexion between rheumatism and chorea cannot be established. But there is more to be said in regard to those examples of chorea to which I have alluded as occurring in immediate con- nexion with acute rheumatism, and in virtue of which it may be ne- cessary to admit that the one disease actually gives rise to the other. In most of these the subjects are not children, but young adults in whom violent and often fatal clonic convulsion (whether chorea or not) arises immediately out of acute articular rheumatism, or interrupts or may even precede it. Such symptoms cannot be dissociated from the other phenomena of the disease which they accompany. They furnish examples, along with those of acute delirium and the various forms of cerebral embolism, of the several nervous accidents to which the condition of the blood during acute rheumatic fever gives occasion. In their origin, therefore, no less than in their character and choice of subjects, these convulsions are to be kept distinct from the chronic disorder of childhood which we are now discussing. It is not to the present purpose to notice other arguments of statistics which are opposed to this asserted connection. These are to be found, as I believe, in the time of life, the sex, and the geogra- phical distribution of chorea and rheumatism respectively, as well as in the fact that while students of chorea are earnestly contend- ing for an alliance of this kind, the students of rheumatism, with their far richer material and ready recognition of complications and sequelae, give no encouragement to such proposals. The doctrine of the rheumatic origin of chorea, however, is too firmly rooted in this country to be much disturbed by such considera- tions. We no longer seek to verify this connection, but are rather occupied in constructing some plausible explanation of it Mean- RHEUMATIC CONNECTION. 43 while the two most prominent and unquestionable facts in regard to the causation of chorea pass unregarded : the fact that alarm or mental disquiet is its commonest, and, it may be, its constant cause, and the fact that female children are in overwhelming proportion its favourite subjects. 44 CHOREA. CHAPTER III. THE HEART SYMPTOMS OF CHOREA. Facts for explanation — The comparative frequency of heart disturbance in chorea of different ages — The heart symptoms referred to endocarditis — to a sympathy on the part of the heart with the muscular disorder — Difficulties in the way of both hypotheses — Conclusions. It is now generally admitted that there are certain heart symptoms proper to chorea apart from those which are due to its association with acute rheumatism or with anaemia. It is admitted as well that cardiac apex murmur is, more often than was at first believed, the sign of a functional disorder on the part of the valvular appa- ratus ; the precise nature of this defective action has been in great measure elucidated, and it has been repeatedly shown both in chorea and other conditions that such murmurs are temporary and innocuous. With this double advance of knowledge, both in re- gard to the natural history of the disease in question, and the pathology of the heart, we may consider the cardiac symptoms of chorea from a new standpoint, with a clearer and more just appre- hension than heretofore of the facts involved, and fuller and more definite material for their discussion. In what is to follow I pro- pose to investigate the present state of opinion as to the nature and significance of the cardiac phenomena of chorea. To place such opinion side by side with the actual facts of the case, and upon this review, to consider whether by means of any existing hypothesis, or of any modification or combination of hypotheses, the question at issue finds reasonable solution. The heart symptom of chorea chiefly discussed amongst us is systolic murmur. In regard to the rate and rhythm of the heart, HEART SYMPTOMS. 45 as well as the precise seat of the bruit, there are, as we shall see, doubts and differences ; but all observers allow that a soft systolic murmur is apt to arise in the course of chorea, independently of rheumatism or of anaemia, which is variable in tone, disappearing and reappearing, often influenced by posture and' by exercise, most audible in the majority of instances at the left apex, productive of no sensation of its own, and indicated by no outward sign of dis- turbed circulation. ^ Between this cardiac murmur and the choreic restlessness there is no equality or parallelism. No particular manner or degree of mismovement, and no particular temperament of patient is especially liable to this heart affection. The choreic murmur comes and goes secretly and without notice, there is no recognisable law of its occurrence, and when auscultation has dis- covered it, neither treatment nor prognosis is greatly influenced by the discovery. But while these symptoms on the part of the heart, in their physical characters, limited duration and freedom from injurious consequences, remind us most of the so-called functional murmurs, yet they have a near relationship to substantive disease. This is not matter of inference, but of direct observation. There is no fact of chorea better established than this : that in the majority of fatal cases — dying either in the course of chorea or shortly after- wards — a fibrinous bead-like fringe is found edging the mitral valve, and occasionally the aortic valve also.^ That this condition is the effect rather than the cause of the chorea, we are compelled to assume, in the numerous instances where murmur has gradually developed in the course of the muscular disorder. There are others, however, and especially those which arise out of acute rheumatism (be they few or many), where the sequence is different, * Such is the general description. Dr. Walshe, however, insists upon the regularity of action, and Dr. George Balfour asserts that the murmur ' while it lasts is constant and unchanging.' See Walshe, Diseases of Heart, p. 89 ; Balfour, Diseases of Heart, p. 166. 2 ' Pathology of Chorea,' Dickinson, Med.-Chir. Trans., vol. lix. See Chap. IV. ' On Fatal Cases of Chorea.' ^6 CHOREA. and the cardiac murmur, from immediately preceding the chorea, may be taken to indicate an endocarditis which is certainly not the consequence and may possibly be the direct cause of the chorea. But although endocarditis, upon this showing, seems to be connected with chorea in a double relationship, both as its cause and its product, there are obvious difificulties in the way of ad- mitting it at all. If endocarditis is really at the origin of chorea, why do the physical signs which should announce it so often lag behind, and why are they in themselves so equivocal and without the usual accompaniments and results of valve inflammation ? If, on the contrary, endocarditis is not the origin of chorea, but arises out of it, by what sort of agency is this accomplished, and why does not chorea, in virtue of this association, lay the foundation of organic heart disease as acute rheumatism does ? Two conflicting doctrines are thus offered for our acceptance, neither of which we can wholly accept, and neither wholly reject. It is certain on the one hand that cases of chorea which happen to die (whether from the severity of the disorder or otherwise) very commonly exhibit a fringing of recent lymph on the mitral valve ; it is not less certain on the other that the non-fatal cases (that is of course the vast majority) very rarely exhibit valvular disease in later life. It is certain that both the auscultation signs and the subsequent history of chorea murmur correspond most closely with the dynamic murmur of young women ; it is not less certain that chorea prefers children rather than young women, and shows after death the actual material of endocarditis. Such are the difficulties which meet us at the outset in en- deavouring to explain the cardiac symptoms of chorea, and it is obvious that the account I have just given in regard to these symptoms does not contain the key to the solution. There still remain, however, other factors of the problem, some which are differently interpreted by different observers, and some which per- haps have not as yet received their due share of notice. Thus, for example, there is the character of the heart's rhythm, which some HEART SYMPTOMS. 47 report to be irregular, and others undisturbed ; and there is the further question as to the mode of incidence of cardiac phenomena in chorea in respect of age. It may be that the discrepancies which appear at present in regard to the precise characters and relative frequency of heart disturbance will disappear when we come to compare patients of similar age. We know, for instance, that the liability to such disturbance is in no direct relation to the violence or the particular method of the choreic attack ; but we do not know that it has no direct relation to the age of the patient. Again, we know that choreic murmur may occur inde- pendently of any marked change in the cardiac rhythm ; but we do not know that this is so at all periods of life, or that disturbed rhythm is not the rule rather than the exception in young children. In what follows I propose to consider the characters and the frequency of cardiac disturbances in young children as contrasted with those who are older ; to compare actual observations upon these points with the more general statement of authors ; and, by the help of this comparison, and the material it will require, to consider what hypothesis serves best to reconcile phenomena which at first sight seem to be conflicting. I take for this purpose, as before, the following : — 1. Dr. Dickinson's seventy-one cases (not 70 as stated) ap- pended to his paper in the fifty-ninth volume of ' Medico- Chirur- gical Transactions,' and referring to children treated at the Great Ormond Street Hospital. 2. One hundred and thirty-two cases of children treated by myself at the same hospital, but at a late stage of their dis- order. 3. Thirty-nine cases of older children and young adults under my charge at the Westminster Hospital. ^ 4. Fifty cases similar to the last recorded by Dr. Owen in the ninth volume of the ' St. George's Hospital reports.' 1 See Appendix B. The last six cases of the second series were added later, and are not reckoned above. 48 CHOREA. These four groups represent respectively, as will be seisn more particularly in the sequel — (i.) Young children from 2 to 12 observed at an early period of their disorder. (2.) Young children of similar age observed at a later period of their disorder. (3.) Older children with a few adults. (4.) Older children, with a considerable proportion of young adults. (i.) In Dr. Dickinson's table the number has to be reduced to 69, on account of two cases with the ages omitted. The children are all young. There is in fact but one, a girl, as old as 13, and but 3 as old as 12 ; the youngest is 3, the next youngest are two of 5 ; the ages most largely represented are 10 and 11 Taking the 69 cases, with a view to ascertain the incidence of heart affection at different ages, it will be found that of the 21 children who are 8 years old and under, only 4 are free from heart affection, 3 of these being of the age of 8 ; while of the 48 children above 8 years old, there are 17 free from heart disorder.^ If, how- ever, we state the numbers somewhat differently, classing the children of 8 amongst the elder ones, we shall then find that in 1 1 children under 8, there is but one free from heart affection, while in 58 of 8 years old and over, there are at least 19 free from any abnormality of cardiac action which can fairly be reckoned. ^ As regards irregularity, the 69 cases give 20 such examples, 12 of ir- regularity alone and 8 of irregularity together with murmur. Of the 21 children 8 years old and under, there are 5 with hearts • Of the 17 children of eight and under having heart disturbmce, as many as five had had acute rheumatism, to which therefore the heart conchtion might be referred. On the other hand^ of the 31 children over eight having henrt disturbance, as many as six had had previous attacks of chorea ; to which period, therefore, rather than to the age retorded, the date of the heart disturbance might be referred. * I have made these calculations from the tables themselves (to which I refer the reader), and they do not precisely correspond with Dr. Dickinson's own sum- mary (on p. 34 of his paper). This latter, however, has reference not to the ages of the patients, but to the several causes of the chorea. HEART SYMPTOMS, 49 irregular only, and 3 with irregularity together with murmur. Of the 48 children over 8 years old, there are 7 with hearts irregular only, and 5 with irregularity together with murmur, I take next a second group of children from the same hospital who were under my own charge, having been received from one or other of my colleagues. The ages are still between two and twelve, as in the first group, but the children come under observa- tion at a later stage of their disorder ; the great majority were chronic cases, some few were convalescent. The total number of these patients was 132 (appearing as 137, from 5 of them having been twice admitted). The age distribution is as follows : — 12 years old are only 2 1 1 years old are 24 9 and 10 years old 47 8 years old and under (36 of these being under eight) ..... 59 132 In the whole 132, 47 (more than a third) have heart murmur or irregularity. The 59 children of eight and under have 24 examples of heart disorder ; the 72 children over eight have 23 such examples. In other words, the proportion of heart disturb- ance is about 40 per cent, in the younger children, and about 32 per cent, in the older ones. Again, if we take the extremes of age in both directions, that is to say, if we compare the 26 children of eleven and twelve (the oldest we have to do with) with 36 who are imder eight, we have 9 of the former having heart disorder against 16 of the latter, the actual percentage of heart disturbance being thus somewhat greater in the younger than in the older children. Of cardiac irregularity, as apart from murmur, not including mere unevenness of rhythm, there are but 9 examples, 6 of these referring to the little children. These cases, therefore, representing the same ages as Dr. Dickinson's, but referring to a later stage of the affection, yield a E 50 CHOREA. much diminished proportion of heart disturbance of whatever kind. It is a little over a third, whereas in the former table it is over two thirds. And what is still more remarkable, irregular action of the heart, largely represented in Dr. Dickinson's list, hardly appears in mine, and is almost confined to the younger patients. It thus appears probable, taking these two tables as they stand, and comparing them together, first, that the heart affection of chorea is not less but more marked in early than in later child- hood ; secondly, that observation of the disorder at a late period discovers less heart disturbance than at an earlier, and especially that cardiac irregularity, a distinguishing feature of the chorea of early childhood, is found at the beginning rather than at the end of the attack. Such, I say, are the conclusions to be drawn from a bare enumeration, and so long as we confine ourselves to children the majority of whom are not over ten, they are conclusions which need no correction. But when we enlarge the view and admit older children and adults ; in other words, when we go from children's hospitals to general hospitals, we can no longer accept the mere figures without some attempt at adjustment on account of disparity of age. Thus, for example, all other things being equal, the greater the age the larger the proportion of heart dis- turbance independent altogether of chorea. We have to consider besides, that in comparing childhood with adolescence, in respect of the signs we are discussing, we have to encounter at the later period those functional murmurs (variously named, and attaching especially to young women) which, although they be altogether distinct from chorea as such, are not in practice dis- tinguishable from it. Add to this that the older the patient the greater the probability that the chorea is not the first, but the second or the third attack, and the heart disturbance therefore but a revival of what has occurred in childhood, according to the well known law of the disorder to repeat itself. Such cases HEART SYMPTOMS. 51' illustrate in fact the liability of early childhood in respect of heart sympathy : they are taken to illustrate the hability of the more advanced age at which the patient comes under notice. Again, some account must be taken of the previous occurrence of acute rheumatism, which is likely to appear in larger proportion in the histories of the older patients than of the younger, and to con- tribute its share of heart murmurs which are not choreic but rheumatic. It would be quite unfair, I say, to attempt the com- parison we are now about without making allowance for discrepan- cies of this kind ; without providing, that is to say, for the fact that the younger the child the more likely the heart disturbance to be part of the chorea, the older the child the more likely to be accidental or contributed. Moreover, in any exact computation as to the age-incidence of heart disturbance, it would be necessary of course to exclude from the comparison anaemic and chlorotic girls, as well as those who in earlier childhood had had either chorea or acute rheumatism. In the case of such children as we have been considering, all of them young, the correction necessary on this account would not amount to much, and we need not stay to make it ; but in the case of the older patients we are now coming to, it makes a very wide difference. It would be altogether delu- sive and misleading to place the old and the young side by side with the view to an even comparison in respect of heart sympathy in chorea. Numerical equality between the two classes, or any- thing near an equality, would indicate, without doubt, that the heart was more often implicated in the chorea of the young than of the less young. How the figures stand in these respects with older patients, and how large a correction is needed for such reasons as have been mentioned, will be seen from the examples of chorea which remain to be quoted, viz., the 39 of Westminster Hospital and the 50 of St. George's Hospital. In the 39 Westminster cases 19 are twelve years old or over, 5 of them being young women. In the 50 cases reported by Dr. Owen in the ' St. George's Hospital E 2 52 CHOREA. Reports,' vol. ix., of two following years, as many as 27 are twelve years old and over, 1 1 of these being young women and 6 young men. We have thus in the four series of cases now rehearsed a constant rise in the ages of the patients. Dr. Dickinson's are the youngest, and the St. George's cases are by much the oldest, the Westminster series standing midway, and my own children's hospital cases differing from Dr. Dickinson's more in the duration of attack than in age of subject. The 39 Westminster cases are distributed as follows : — 13 years and over (two being young women) . 15 12 years old 4 1 1 years old 5 9 and ten years old 9 ] 8 years old and under 6 39 Of the whole number, 13 would have to be excluded, 5 on account of acute rheumatism at some earlier period, and 8 on account of previous attacks of chorea. The remaining 26 are made up of 19 who have no cardiac disorder, and 7 who have either murmur or irregularity. Of the 19 who have their hearts free, 15 are over eleven years old ; of the 7 who have cardiac murmur or irregu- larity, only I is over eleven. In the two tables of cases of St. George's Hospital there are 15 examples of heart disturbance in 50 cases, 10 of these 15 are found among the 27 cases over twelve years old ; only 5 are found among the 23 cases under that age. Upon this showing, it would seem that cardiac symptoms in chorea are not more rare but much more common after twelve years old than before. Upon examination, however, of 10 individuals exhibiting heart murmur and over twelve years old, it appears that 4 of them have had previous attacks of chorea, i had pericarditis while in hospital, I developed murmur just after acute rheumatism, and 2 were weakly girls, aged fourteen and fifteen respectively. There are tl\us 8 of the 10 whose murmurs are accounted for apart from HEART SYMPTOMS. 53 chorea altogether. We get but too properly choreic murmurs in 2 7 choreic patients of ages ranging from thirteen to eighteen, as large a proportion, I am disposed to think, as will be commonly found at that period. There is indeed considerable difficulty in fixing the time of life when the liability to heart disturbance in chorea finally ceases. The evidence already adduced shows, I think, that childhood is particularly liable to it, and that irregularity of rhythm distin- guishes this earliest liability, whether with or without systolic murmur. It is also generally agreed that the rare chorea of ad- vanced life has little connection with heart disturbance. But of the middle period our knowledge is far from precise. For it so happens that at the very time when, with the progress of develop- ment, the frequency of cardiac murmur in chorea is obviously lessening, there comes a period of life (and especially with young women) when the precise significance of the symptom we are discussing is hardly discoverable. Murmur may be choreic or (so-called) anaemic. It would seem impossible at such time to determine with any approach to certainty the proper liability of the heart in connection with chorea. We know only that so soon as this interval of obscurity has passed, and, with the com- pletion of adult life, the indications furnished by the heart are again trustworthy, cardiac sympathy in chorea is hardly recog- nisable. From the facts now adduced several conclusions seem to follow. Heart symptoms in chorea occur most frequently during a period which is included between two years of age and about seven or eight. The age of greatest liability to choreic heart disturbance thus nearly coincides wdth the age of greatest liability to chorea. Again the abrupt decline in the liability to chorea, which occurs at about fifteen, corresponds with a similar decline — if it be not rather a cessation — in the disposition of the heart to share in the disorder of the voluntary muscles. It has been already observed that irregular cardiac action, 54 CHOREA. either alone or preceding, or accompanying the murmur, is a pretty frequent feature in the younger children of Dr. Dickinson's table. In my own children of similar age, but in a later stage of chorea, it is much less common. In the Westminster cases, where the patients are older, it is also rare, and in the St. George's cases, older still, irregularity hardly occurs. This particular symptom in the younger subjects is often the single sign of the heart's affection. I believe, although I cannot show by tables, that there are very few little children, if there be any, in any early stage of chorea, who do not exhibit a marked unevenness or inequality of cardiac rhythm. Such unevenness will sometimes develop into murmur and sometimes not, murmur which is almost always variable with the child's position, sometimes best heard over the pulmonary cartilage rather than at the apex,^ apt to come and go, and often finally lost sight of before the chorea itself has disappeared. The second pulmonary sound is seldom accentuated or doubled. These statistics in regard to the age-incidence of the heart symptoms of chorea enable us to render a fuller account than heretofore of the particular phenomena. Dr. Walshe's assertion that the cardiac action is regular must be limited to the older subjects, or to the later stages of the disorder. Dr. Bristowe's statement that the greater number of choreic patients have some cardiac defect, irregularity amongst the rest, must be qualified by adding that this large proportion refers to children, and that ir- regularity as a sign of heart disturbance is almost confined to them. ' The fact of mitral regurgitation being most audible in this situation, is well explained by Naunyn, as reported by Rosenstein (Ziemssen's Cyclopcrd., vol. vi. p. 122). He points out that the second intercostal space, close to the left edge of the sternum, coincides with the point of the left auricular appendix, which winds round the pulmonary artery and lies in front of it. Now with the abnormal current of blood flowing towards the auricle, we can easily understand, says Rosenstein, ' how the sound should be conducted to the spot mentioned better than to- wards the apex, more especially in cases where the appendix is long enough to lay its point against the anterior wall of the thorax.* HEART SYMPTOMS. 55 Thus the several factors of the problem with which we have to deal summarily stated are these : — 1. In the course of the chorea of childhood the heart is apt to become irregular or uneven, and its first sound to be followed by apex murmur, which is variable in pitch, influenced by posture, seldom audible in the axilla or at the angle of the scapula, and which disappears along with or shortly after the chorea, the heart and the circulation suffering no injury. 2. This liability on the part of the heart, to what, from its signs, would seem to be a functional disturbance, is independent of the violence or method of the chorea, but dependent upon the age of the patient, the younger children being the most, and the elder the least liable, while beyond childhood there is little if any liability of the kind. 3. These heart signs of chorea — acute rheumatism being ex- cluded — give rise, as a general rule, to no symptoms whatever affecting the health or comfort of the child. They make no ap- parent difference to the prospects of recovery or the structural in- tegrity of the heart. Nevertheless, choreic children having this murmur and happening to die, either with or shortly after recovery from chorea, very commonly exhibit a beading of recent lymph on the mitral valve. Such, I say, are the chief statements which statistics seem to warrant. I will venture to add another, which, so far as I know, . has never been statistically reckoned, but which no one will gain- say. It is, indeed, the most constant of all the heart symptoms of chorea, and met with at a later age than the rest I mean the acceleration of the heart and pulse. ^ 1 Ziemssen says ' cases of arhythmia are certainly very rare, and there is hardly anything published about it. ' On the other hand, he regards acceleration of the action of the heart as common, and acceleration of the pulse as quite constant, although, as he believes (I am convinced erroneously), the bodily heat is not changed even in severe cases. — Ziemssen's Cyclopced. art. Chorea, p. 436. The heart symptoms mentioned in the text are not the only ones to be met with in chorea. I have heard presystolic murmur and both accentuation and doubling of the second sound, and this in cases where there was sufficient evidence 56 CHOREA. Now there are two hypotheses of the heart phenomena of chorea, each of which has special regard to certain of the facts just mentioned, although neither is able to reckon with them all. The two are in a sense mutually antagonistic, inasmuch as what the one rejects the other prefers. Both theories have their sup- porters in this country. The one asserts that the heart symptoms of chorea are due to endocarditis, the precise relationship of the muscular disorder to the valve inflammation being variously in- terpreted. The other asserts that the cardiac signs are intrinsic, a part of the chorea itself That there is some evidence in favour of each of these views we have already seen. The task before us is to reconcile them. To the belief that the heart murmur of chorea is due to endo- carditis I know of nothing in the actual clinical symptoms that can be opposed. Our information in regard to the physical signs of endocarditis per se is, indeed, vague enough,' while the pro- bability of its occurrence in chorea is directly favoured, as we have seen, by post-mortem evidence. The distinction between organic and functional heart murmurs, founded on hard and fast lines as to the transmission of the bruit, is of doubtful validity. * A well-pronounced functional murmur,' says an excellent ob- server,^ ' may be as diffused and transmitted as an organic' ' A systolic apex murmur,' says Dr. Bristowe, ' is a positive proof of that the heart disturbance was purely choreic. But I am not able to say at present to what ages these exceptional signs chiefly attach, or what is their proportion to the commoner symptoms. ' ' There are few diseases,' says Rosenstein, ' the presence of which is diagnos- ticated so arbitrarily as that of original acute and subacute endocarditis.' He proceeds to point out the necessity of ' proving the existence of other symptoms in addition to the mere murmur, especially intensified second sound in the pulmonary artery, the localisation of the murmur, or transverse hypertrophy of the cardiac volume.' — Rosenstein, art. Endocarditis, Ziemssen's Cyclopccd., vol. vi. Dr. Bristowe says : ' If in the progress of any of those diseases of which endocarditis is a common complication, we detect a recent cardiac murmur, and if further observation proi.'e<; this to be a permanent phcnomcJion, we cannot reasonably doubt that endocarditis is present." — Bristowe, Theory arid Practice of Medicine, and edition, p. 522. 2 Dr. Nixon in Dublin Journal of Med. Science, vol. iv. p. 575. HEART SYMPTOMS. 57 regurgitation.' We have, indeed, just as much evidence, so far as physical signs are concerned, of endocarditis in the course of chorea as we have of it in many instances of acute rheumatism. In both cases ahke a soft and not conveyed murmur may be proved, in the event of death, to depend upon valve deposit, and in both alike (I do not say to a like extent), when death does not so intervene, the murmur has been found to disappear. In frank recognition of these facts. Dr. Wilks expresses the belief that all mitral systolic murmurs associated with chorea are organic ; and Dr. Sansom,^ in a short summary of the prevailing opinions upon the subject, admirable for its conciseness, arrives at a like con- clusion. But when this much has been granted and endocarditis is admitted, how much are we the better? What is the mode of origin and what is the sequel of such endocarditis? It is but exchanging one difficulty for another. For we have to suppose, upon this hypothesis, not only that endocarditis may arise in and out of chorea, and that the younger the child the greater the pro- bability of this event, but also that cardiac irregularity sometimes precedes and sometimes takes the place of regurgitation, whilst none of the injurious after consequences which attend endocarditis in its other relations are found to ensue here. In face of such grave objections, we are forced to admit that, although physical signs do not contradict it and post-mortem evidence may seem to be directly in its favour, the theory of choreic murmur (not to speak of the other signs) which invokes endocarditis is too difficult. It is inconsistent with all that we know of that inflammation in its various pathological relations ; and apart from these we know remarkably little about it. It is in fact by virtue of this vague know^ledge that endocarditis maintains its hold in this connection. A form of heart inflamma- tion special to chorea, and having a history and mode of termina- tion of its own, is a creation of too shadowy a kind to be easily 1 Sansom, Diseases of the Heart, p. 89. 58 CHOREA. dealt with. But so soon as anything definite and within reach of investigation is alleged in regard to it, when, for instance, it is said (as Trousseau, Bouillaud, Roger, and other French authors say) that the endocarditis of chorea is in fact rheumatic, although the rheumatic element is itself latent, we have a distinct yet still but partially tangible issue. Of the relationship between chorea and rheumatism I need not speak in this place. I have endeavoured to show elsewhere that although real it is rare and exceptional, and both Walshe and Hayden are of the same opinion. However this may be, no one will refuse to admit that the cardiac symptoms of chorea are observable someiifnes when there is no open rheumatism either past or present. Now if in all such instances we are to say, in the first place, rheumatism is actually present, but after a secret and ineffable manner, and in the second place endocarditis is the consequence of that rheuma- tism, but it is an endocarditis which is exceptional in commencing after the chorea has begun and disappearing along with it, we account for the facts no doubt, but after a fashion which rather begs the question than provides for it. We are thus compelled to regard the heart symptoms of chorea, taking them in their entirety and not by arbitrary selection « — the early irregularity, the variable mitral murmur, the very fre- quent acceleration of heart and pulse with frequent fluctuations in the rate of both — as being altogether special and peculiar. They are signs of the heart's sympathy with the voluntary muscles, and are seen most at that early time of life when the antecedence of acute rheumatism is the least probable. The heart suffers, by whatever mechanism or nervous influence, part and parcel with the rest of the muscular system, or rather it is apt to suffer. Such a conclusion, I say, in some sense or other, seems self-evident, and is indeed involved in the statement that the heart symptoms of chorea are met with in no other association. Obvious and inevitable as it may be, it is far from being easy of application. For if it be granted, in the words of Dr. Walshe, HEART SYMPTOMS. 59 ' that the apex murmur of chorea is plausibly ascribable to dis- ordered action of the muscular apparatus connected with the valve,' why does not the same disordered action extend to the ventricular walls ? It may be answered (although Dr. Walshe does not admit as much) that in young children there is both disorder of rhythm and regurgitation ; but the answer is insufficient. These two signs do not commonly concur nor do they vary together. And it is not young children alone that we have to consider, but the elder patients as w^ell ; and in these latter, while murmur is not infre- quent, any marked irregularity of cardiac rhythm is certainly rare. Thus we have to suppose that the fibres of one and the same muscle are acting regularly as regards the ventricular wall, but irregularly as regards the papillary muscles. ' We should be forced to admit,' say Dr. Hayden, ' that whilst the greater portion of the length of certain muscular fibres contracts with perfect order and regularity, the remaining and smaller portion of the same fibres act spasmodically and out of harmony with the former. ' ' No physio- logist,' he adds, ' bearing in mind the unity of nerve centres and the community of nerve distribution enjoyed by both portions of the same fibres, would admit such a doctrine.' ^ There are other objections equally formidable. It is not in the nature of chorea to dissever the normal combinations of muscular movement. It stirs the very same muscles that are employed by purpose or emotion, and moves them in a similar way, only less precisely and with less definition. Apart from anatomical considerations, therefore, a choreic spasm of the heart, which should select the papillary muscles and leave the cardiac walls, would be untrue to the pattern of chorea elsewhere. And again, if this choreic conduct of the heart be but an extension of the same disorder which affects the other muscles (an extension, as Dr. Kirkes points out, to which there is no parallel in any other involuntary muscular organ), the most violent and general chorea ought to exhibit this cardiac disturbance the most. But it ^ Diseases of Heart and Aorta, p. 268. 6o CHOREA. is not so. On the contrary, the violent chorea of puberty has seldom either cardiac murmur or cardiac irregularity, while the chorea of the young child, which is very rarely violent and is sometimes rather a paresis than a disorder of movement, exhibits both these forms of heart affection very often. And even if these difficulties should be overcome or the ground of some of them disputed, it would still, I think, be hard to understand how the great centre of circulation and (in one sense, at all events, within the experience of everybody), of emotion should become the seat of a mismovement at all resembling the choreic contortions of the visible muscles without giving rise to sensations of grave disquiet, sensations, be it remembered, which, so far as young children are concerned, the favourite subjects of such cardiac disorder, are conspicuously absent. It would thus appear that both the hypothesis which supposes endocarditis to be the cause of choreic murmur and the hypothesis which supposes an extension of the muscular infirmity to the heart to be the cause of it are fallacious. Both theories commit us to too much. And if, in this strait, we have recourse to vaguer ex- pressions having regard to the ' impoverishment ' or ' vitiation ' of the blood, we neither provide an adequate explanation nor allege a demonstrable cause. But while thus compelled to abandon one position after another, we must beware lest by too precipitate a retreat we quit ground which it is essential to hold. We may even venture so far upon the assured facts of the case as to make some sort of stand and to affirm something. If the views just announced cannot be held in their entirety they must be held with some modi- fication or other. There is no alternative. Part of the perplexity arises, as has been shown, from a consideration of the harmlessness, variability, and limited duration of the choreic murmur. But if these facts are in themselves secure we may rely upon them to force at least this conclusion, that the conduct of the heart in chorea is similar to its conduct in anaemia. Whatever explanation, therefore, applies best to the precise mechanism of the so-called functional HEART SYMPTOMS. 6 1 or dynamic murmur cannot be altogether foreign to the circum- stances of chorea. It is now generally admitted that the murmurs once called haemic, anaemic, and by similar names, implying dependence on some blood change, are much more often than was at first supposed produced by actual regurgitation, due to a temporary defective action on the part of the mitral valve. Such murmurs are to be heard in purpura, in anaemia, in the general muscular relaxation due to nervous strain, in epilepsy, as well as in typhus, and enteric fever, and even in simple pyrexia. This dynamic mitral bruit may, by exception, be harsh and far-sounding, audible in the axilla and even at the lower angle of the scapula, yet never- theless by its ultimate disappearance give ample proof that what- ever may befal the valve during its continuance (and there is some reason to believe that a material change actually occurs) is not abiding or permanently mischievous. So much, I think, is gener- ally admitted. The question for us now is, whether the same explanation of the mechanism of this murmur which applies to its association with the conditions just mentioned applies also to its association with chorea. No one, so far as I know, has more ably discussed this subject than Dr. Nixon. ^ His exposition of the various ways in which dynamic apex murmur may arise, and of ' the perfect unity and correspondence in action of the fibres of the walls of the ventricles and of those which are connected with the valves, which is needed in order to ensure complete closure,' suggests to him the inquiry whether this nice correspondence of movement might not be so disturbed by altered nutrition or innervation as to render such closure for awhile defective. The tremor of the voluntary muscles, which is apt to follow upon their violent exercise, is adduced as being analogous to this disordered cardiac action. Dr. Nixon con- cludes that functional mitral murmur results from ' this want of correspondence between the fibres of the ventricle, which obliterate 1 Dublin Journal of Medical Science, vol. Iv. p. 572, &c. 62 CHOREA. the cavity, and those which close the valve ; ' an altered function due, as he believes, ' to some defect in the vital power or condition of the heart itself, which leads either to atony of the papillary muscles or derangement in the rhythm of their movement.' In the further observations of the same author as to the means of separating this murmur from the organic, he alludes to its changeable character, alteration or even disappearance with the upright posture, and the absence (in most instances) of accentuated or double second sound. Thus, in all its essential features, the murmur which Dr. Nixon describes corresponds with that of chorea ; and, except in cases of temporary debility, aucemia and certain conditions already particularised, commonest in the female sex, it is seen in no other connection. Dr. Hayden, assenting in part to these views, would attribute the dynamic murmur not so much to a want of correspondence between muscular fibres as to atony or parietal debility of the walls of the left ventricle.^ Weakness and relaxation of the papillary muscles, he admits as a possible cause of mitral regurgitation. Spasm, or irregular action of the same muscles, he does not admit as a possible cause of it. This regurgitant murmur, ' caused by atony and partial yielding of the walls of the left ventricle at the acme of systole,' Dr. Hayden has long associated with anaemia, purpura, and excessive use of tobacco. His later observations lead him to include chorea and fatty heart in the same category. But the latest and least reserved expression of the opinion that dynamic murmur is in fact dependent on muscular paresis is that of Immermann.* ' The muscular tissue of the heart,' says he, 'owing to the altered state of the blood, is easily fatigued, and this lia- bility to premature fatigue extends to the papillary muscles con- nected with the auriculo-ventricular valves. After any undue exertion of the cardiac muscle a temporary paresis of the musculi papillares ensues. In conscfiuence of this the valve flaps intrude ' Hayden, loc. ctt. p. 274. * Ziemssen's Cyclop., vol. xvi. p. 399. HEART SYMPTOMS. 63 into the auricles with every ventricular contraction, that is, a transient functional insufficiency of the tricuspid and mitral valves is established. As the organ regains its normal energy these grow fainter and ultimately disappear.' He goes on to describe the fatty degeneration of the papillary muscles which sometimes en- sues upon simple anaemia, and which explains both the occasional permanence of these murmurs and their occurrence in connection with fatty heart. It will be admitted, I think, that both the time of appearance and precise manner of the choreic murmur, as well as its mode of origin and duration, varying intensity and limitation to the period of youth, are all nicely provided for in the hypotheses just re- hearsed. If only it be granted that the heart's disorder is really a sympathy with the muscular disturbance, an inexact and precarious correspondence between the ventricular contraction and that of the papillary muscles would seem to serve for chorea even more than for the conditions to which Dr. Nixon applies it. Still better, and without making any assumption as to the heart's sympathy, might we admit the doctrine of partial paresis of the valve appa- ratus, and insist that the excessive muscular movement of chorea is the precise condition w^hich, upon the hypothesis, should give rise to murmur such as we hear in token of fatigue on the part of the heart Adopting either of these views, we might say with Ziemssen that ' change in the valvular sound is a priori more likely in chorea than in any other affection.' ^ But the matter is not so easily settled. Let it be granted that the choreic murmur is due to a functional disorder on the part of the mitral and probably also of the tricuspid valves, by means of which a temporary and variable regurgitation is permitted (and I think so much of admission is inevitable), we have to ask, in the next place, why this regurgitation, whether it be due to faulty adap- tation of the several parts of the heart to one another, or to the paresis of fatigue, is not in harmony with the other symptoms ? ^ Loc. cit. p. 440. 64 CHOREA. If the murmur betokens a sympathy on the part of the heart with the disordered action of the other muscles it ought to appear when chorea is the most generalised ; if, on the other hand, it betokens a fatigue paresis it ought to appear when the chorea is most vio- lent and prolonged. But it does neither. The sympathy of the heart (if it be rightly so called) goes not with the manner or the degree of the chorea, but, as has been shown, with the age of the patient. And, moreover, we should be taking a very partial view of the subject were we to limit the heart symptoms to cardiac murmur. This, we have seen, is but one, and not even the chief, symptom of choreic heart disturbance. There is besides the unevenness or inequality which belongs especially to early child- hood, and, most constant of all, there is the accelerated pace of the heart. Moreover, we have not merely to account for the presence of these additional signs, we have to fit them into their places, and to show why the heart, seldom failing to share the muscular disorder in one way or another, exhibits this mode of disturbance or that according to the age of the subject. We may accept either account of the mechanism of dynamic murmur, and without much violence apply it to suit the circumstances of chorea: that is a little gain no doubt. But when that is done there remain these other symptoms, which, unlike valvular murmur, can com- pare with nothing to be found in other analogous conditions, unless indeed the cardiac unevenness and its acceleration remind us of what is sometimes met with in hypochondriacs and hysterical women, the least promising subjects to throw light upon obscure places in pathology. It would seem then that to understand the conduct of the heart in chorea we must look to chorea itself and the modifications it exhibits at various ages. Here is an affection which both in absolute frequency and in its tendency to implicate the heart, attaches itself to young children most of all, to older children some- what less, to adults hardly at all. At each of these periods chorea, both in the limbs and at the heart, shows itself after a particular HEART SYMPTOMS. 6$ manner. If, then, the action of the heart in this disorder is throughout in real harmony with that of the voluntary muscles, the clue to the secret we are in search of must be sought in the special characteristics of those periods of Hfe to which chorea especially attaches — characteristics, that is to say, in which they differ from infancy on the one hand and adult life on the other. If the reader is not already wearied by the length of this exordium and the some- what circuitous path he has had to pursue, I would venture in such space as remains to take a final survey of the subject from this point of view. I would observe, in the first place, that the chorea of the post- infantile period is the immediate successor of the muscular spasm or convulsion of the infantile period. At an age varying with the ' forwardness ' of the child, the one Hability takes the place of the other. The very same material cause (as for example, intestinal irritation, or the troubles of dentition) will excite convulsion in infancy, and chorea a little later. Again, the chorea of early child- hood will sometimes commence with convulsion, or the two affec- tions may alternate, the same limbs being affected in both cases. Little, of course, can be certainly known as to the actual sensations of the child at this early age, or of the mode in which physical pain gets mentally interpreted \ but it depends probably in some measure upon the degree of completeness of such interpretation whether the response shall be by way of convulsion or by way of chorea. However this may be, the general muscular restlessness of the young child which passes for chorea is not only in close union with spasm in the way just mentioned, it often bears a direct resemblance to it in form. There is an universal unsteadiness in which the muscles of the abdomen and of respiration share as well as the diaphragm and larynx. This generalised chorea which thus succeeds to convulsion is, so to speak, the simplest form of the disorder, without variety, so far as the movements of the several parts of the body are concerned, and without any mental admix- ture. It is an aggravation of the natural unsteadiness of child- 66 CHOREA. hood, an universal and equable overmovement or mismovement extending to all those regiens which it is the nature of chorea as a motor disorder to affect, and it is this merely. There is no inco- ordination, for the muscles have not yet learnt to act together ; there is no distinction between the movement of one part and another, for the several muscular uses are not yet differentiated, and the limbs are stirred indifferently without method or purpose. Such general, featureless agitation of the body is the proper re- sponse to a source of irritation which appeals to no one part in particular and has no mental representation. With maturer age, the bodily movements become varied and definite, and each department of the muscular system sets about its separate business. And as with movement, so with mismove- ment. The chorea of later life is a definite deformity, which alters and disfigures the natural carriage in this way or that according to its seat. It selects those muscles which are the readiest to respond to emotion, and moves them as they are wont to be moved by the passions and employments of life. Thus the choreic distortion of the face is one thing, of the legs another, of the arms another ; and this separateness becomes the more distinct as the individual grows and his individuality, as it is called, becomes more and more marked. The mismovement of chorea betrays the age of its subject, no less than does the natural movement ; and, as life goes on, chorea becomes more and more distinctly localised. In its latest form it is a partial disobedience or misconduct of certain muscles, rendered inveterate by habit, and incorporated, as it were, with the rest of the bodily move- ments. My conjecture is that the conduct of the heart in chorea is in strict accordance with these successive modes or differentiations of the muscular disorder. The heart sympathises with chorea, in one way or another, throughout life, or at any rate until that late period is reached when the affection is both rare and difficult to identify. Not to speak, for a moment, of the valvular murmur, HEART SYMPTOMS. 67 the choreic heart is unrhythmical or irregular in early childhood,^ accelerated in youth, and left undisturbed in old age. Thus, the earliest period of heart sympathy, that of cardiac irregularity, with or without acceleration, corresponds with that chorea which is a purely motor disorder, the earliest transition from infantile convul- sion, and differing from it mainly in the character of the movement, and the implication of the emotional centres ; but resembling it in its exciting cause, wide range, and conformity everywhere to a single pattern. In the heart, as well as the limbs, in the tongue, the speech, the diaphragm, in all the parts, in fact, accessible to emotion, yet without any mental representation of emotion, the choreic disturb- ance of little children is a purely motor disorder, excited very often, like the earlier convulsion, by a material irritation. In like manner, the cardiac acceleration of youthful chorea, with or without murmur, corresponds with that form and period of the affection when the several muscular movements have been differentiated, and there is no longer a single and uniform re- sponse, but each department of the body liable to chorea responds after its own manner to a disorder which is now first mentally apprehended and intimately connected with emotional exaltation. As to the limbs, there is no longer a mere restlessness impartially distributed, but complicated disorder of movement, in which paresis and inco-ordination are variously blended, and which selects, by well-marked preference, those muscles which are the readiest exponents of emotion. And as to the heart, in place of disturbed action and rhythm, there is the proper emotional response with which everyone is familiar in his own person — an acceleration, namely, and often knocking impulse as well. Now, at both the periods we are now considering, in child- 1 Ziemssen's suggestion as to ' the influence of restless muscular action upon aortic pressure and the working of the heart ' {loc. cit. p. 437), fails to apply, I am convinced by repeated observation, in explanation either of the heart's irregularity or of its rate. It is. indeed, at once negatived by the admission that the heart disturbance of chorea is in no direct proportion to the violence of the muscular disorder. F 2 68 CHOREA. hood and in youth, both with cardiac irregularity and cardiac acceleration, we have, or we may have, apex murmur. It has been shown already that for such murmur no other explanation applies except the functional one ; and indeed, in the case of choreic patients, at that time of life when the so-called anaemic murmur is common, I know not how it is to be determined whether valve defect is due to chorea or to anaemia. The same causes may operate in both cases, and the explanation of Dr. Nixon of faulty adjustment of valve and ventricle may serve for both. But there are still the children to be considered ; and, if we are still to apply the argument that the heart symptoms of chorea are in constant harmony with the rest of it, it is necessary to remember that there is another element in chorea besides those that have been alluded to. I mean paresis. The real character of this choreic paresis has been somewhat obscured, I venture to think, by the prominence that has been given to those examples of it which have chanced to affect one side of the body only. I would only remind the reader, in this place, of what has been said before in reference to that strictly localised paresis which mixes with choreic restlessness in children. Sudden both in its access and departure, it may be recognised especially in a droop of the wrist, or a falling of one shoulder, or feebleness of grasp of one hand. It selects this part or that, according to no known rule, and may in some instances show so prominently as to give the patient the appearance of paralysis rather than of chorea. Now, all that can be said of choreic paresis, as we see it in the limbs, may be said as well of choreic murmur, as we hear it at the heart. Both are characteristic of childish, or, at least, youthful chorea ; both are sudden in their development, variable in degree, and apt to come and go without any other corresponding change in the character of the disorder. If, then, weakness and relaxation of the papillary muscles be admitted as a possible cause of mitral regurgitation, and if a HEART SYMPTOMS, 69 debility of this kind may arise temporarily, from tobacco-smoking or nervous exhaustion, much more should it arise in chorea, where — apart from muscular fatigue — there is a special liabiUty to paresis extending, as it would seem, to all the muscles which the disease is able to effect ; and observe that this relaxation on the part of the papillary muscles, which is common both to anaemia and chorea, and productive of regurgitation in both, is not of the same origin in the two cases, nor always accompanied by precisely the same signs. In anaemia it gives rise to an equable murmur, which only ceases with the gradual recovery of the patient ; but in chorea it is (or it often is) a variable and incon- stant murmur, which hardly repeats itself precisely for two con- secutive beats, and which appears and disappears without apparent cause, as does the similar paresis of the voluntary muscles, with which indeed, as I have repeatedly noticed, it may be strictly synchronous, both in its time of arrival and duration. Moreover, with a murmur having this origin, we are rid of the difficulty which attaches to the doctrine of a fatigue paresis, and would require that the valvular defect should be in direct relation to the dura- tion and severity of the chorea ; which would associate it, there- fore, not so much with childhood, as with that violent form of chorea which is commonest at a later period of life. Yet still the old objection will recur. Whence comes it that a temporary paresis of the valve apparatus is found to be associated after death, if not with endocarditis, at all events with a deposit of recent lymph on the edges of the mitral valve? That this appearance does not represent a true inflammation of the endo- cardium is rendered probable by its bead-Uke arrangement, and limitation to the margin, rather than the auricular surface of the valve. There is never ulceration ; and, where time has been allowed to elapse between the chorea and death, the valve is found to be in a normal condition. Only in cases where death supervenes either in the course of the chorea or very shortly after- wards is this appearance met with, and in these not always. There 70 CHOREA. is ample clinical and post-mortem evidence that it is neither lasting nor injurious.^ Mindful of this strict limitation of the facts to be interpreted, and of the necessity, in the last resort, of reaching a solution upon the principle of exclusion, the question suggests itself whether an imperfect closure of the mitral orifice, or rather such uncertain action of the valve segments as closes it at one time, but not at another, might not, at the approach of death, and when, with the flagging action of the heart, fibrine is very readily deposited — so determine the seat and method of such deposit as to produce, at the edge of the valve, the bead-like appearance that we see ; the condition which immediately precedes death being one of the essential factors of the occurrence. It may be said, indeed, that whatever is true of the dynamic murmur of chorea must apply equally to the same murmur in anaemia and elsewhere. But of the post-mortem appearances of simple anaemia we know absolutely nothing. There is, how- ever, a disease having a striking likeness to chorea in respect of its preference for the female sex, near connection with hysteria and with mental emotion, and apparently anomalous blending of functional with organic disturbance. In exophthalmic goitre we have at first acceleration of the heart and afterwards mitral mur- mur, a murmur which sometimes wholly disappears with the com- plete recovery of the patient, and sometimes is the precursor of organic mitral disease. In conclusion, I would briefly recapitulate the facts which it has been the object of this paper to establish, together with the hypothesis which they seem to support. The facts are that in chorea the heart is apt to sympathise with the voluntary muscles ' See Chap. IV. p. 78. The suggestion in the text is in fact that of Dr. Dic- kinson. ' The beads,' he writes, ' are usually confined to the inner surface of the mitral valve and arranged along the attachment of the thin edge, where a line of minute but abrupt prominences is presented to retrograde blood, but an arrange- ment of more gradual slopes to blood flowing normally. Thus possibly the collection of fibrine is the consequence not the cause of the regurgitation.' — Pathology of Chorea, loc. (it. p. 37. HEART SYMPTOMS. 71 at all ages up to the adult period, this sympathy being shown as well by dynamic murmur as by accelerated action, unevenness of rhythm, and, not seldom, the excited impulse common in hysteria, the particular manner of response being dependent upon the age of the patient. The hypothesis is that these several modes of heart affection correspond with as many modifications of chorea, which are exhibited not in the heart only but in the voluntary muscles as well ; these several regions sharing jointly, each in its own degree and after its own manner, in a disorder the area of whose influence is co-extensive with that of ordinary emotional disturbance ; and, particularly, that in all such variations the motor element of the affection is represented mainly by in- equality and unevenness of cardiac rhythm, the emotion element by acceleration, and the paresis element by dynamic murmur. 72 CHOREA CHAPTER IV. CHOREA AS A FATAL DISEASE. Recorded examples of fatal chorea — Age and sex in relation to fatality— Exciting causes — Morbid anatomy — Conclusions. The pathology of chorea needs to be discussed upon a wider basis than that which is offered by morbid anatomy ; nevertheless, no survey of the disorder can be considered complete which fails to take into account the evidence to be derived from fatal cases. The review of a sufficient number of examples of this sort, apart from any direct discovery of morbid change, cannot fail to be of service in exhibiting the age, sex, and temperament most liable to succumb to such attacks. We shall be thus assisted in separating from one another a highly dangerous form of convulsion, and that far commoner motor disorder which, whether violent or not, has little or nothing of danger about it, and probably recovers best without active medical interference. Yet with this obvious service to be got from the study of fatal chorea, its occurrence is so exceptional, that no single observer from his own experience can do more than contribute a very small share of the material necessary for the purpose of drawing any general conclusions. Thus, Dr. Hughes, in so large an hospital as Guy's, was able to collect from all the records by various hands, which had been preserved during more than thirty years, only eleven fatal cases. Dr. Dickinson, from a similar re- cord at St. George's, extending over thirty-three years, collected sixteen such cases, while the large field of observation offered by the Hospital for Sick Children during fifteen years furnishes but AS A FATAL DISEASE 73 six. These examples, it must be understood, include those dying with chorea as well as those dying of it. Although it is not always easy to distinguish between these two classes, it is certain, as will presently appear, that the cases of death attributable directly to chorea fall very far short of the numbers just given. It must be added that many of the earlier records are very defective, that there is no uniform plan of tabulation, and that with different ob- servers different points of interest have received mention. On the whole, it may be said that if all the recorded accounts of fatal chorea in this country which are fairly complete and readily accessible were put together, the total number would not be large, and that in a considerable proportion of these the immediate cause of death and the choreic disorder would be found in very remote and uncertain connection. Such being the facts of the case, I have thought it of interest to bring together 80 cases of death in connection with chorea, or at least with convulsions resembling and called chorea. They are made up as follows : — {a) 3 cases (quoted by Dr. Bright), Med.-Chir. Trans., vol. xxii. ; {b) 11 cases (Dr. Hughes), Guy's Hospital Reports, 1846 (first series) ; {c) 7 cases (from same source), 1855 (second series) ; [d) 34 cases (Dr. Tuckwell), Bartholomew's Hospital Reports, vol. v. ; {e) 22 cases (Dr. Dickinson) Med.-Chir. Trans., vol. lix. ; (/) 3 cases (Dr. Peacock), St. Thomas's Hospital Reports, vol. viii. With these references and the frequent mention I shall have to make of each of these eminent authors no one can quote against me on their behalf the line that Virgil wrote on the palace gate. The present object is to bring together under one point of view a body of evidence derived from many sources without ad- mixture of extraneous matter. Of the conclusions to be drawn from such evidence the reader will judge for himself. The points I propose to examine concern the age and sex and common exciting cause of fatal chorea, together with its most obvious morbid associations. It will be seen immediately that 74 CHOREA only a proportion of the 80 cases are given in such detail as to be dealt with in all these respects. Thus, Dr. Tuckwell's account is no more than a short summary occupying one paragraph of his paper just quoted. Dr. Hughes's first series is described by him- self as ' very meagre and imperfect ; ' Dr. Bright's 3 cases, although so often quoted, are not altogether in point ; in one there was trismus and tetanic spasm, and in a second paroxysmal attacks described as hysteric. In the several enumerations now to be made, therefore, while 80 will be the extreme number dealt with, smaller numbers will have to serve in regard to particular points where the information is defective. With such deduction the cases available in all respects will not exceed 30, but 30 in the circumstances of this disorder is a large number, it is the gleaning of a very wide field, the sum of fatality out of many thousand examples of chorea extending over a long period of years. Taking in the first instance the gross number 80 as repre- senting so many individuals dying in connection with chorea, the most striking point of all to those who are in the habit of regarding chorea as a child's affection is the large proportion of adults and the small proportion of children. Of the 80, 48 are over thirteen years of age, and the ages of two are not stated. Excluding Dr. Tuckwell's numbers, where further par- ticulars are not given, we have in 46 fatal cases 27 who are over thirteen and nineteen who are under that age. Of the 27 over thirteen, as many as 23 may be said to have died of chorea. Of the 19 children, 10 at the utmost died of it, the youngest of these being a girl of seven (No. 19 in Dr. Dickinson's table, loc. cit). Or, again, excluding Dr. Hughes's first (and defective) series, we should have, out of a total of 35, 20 fatal cases over the age of thirteen, of whom 16 died apparently of chorea ; and 15 under thirteen, of whom only seven died ai)parently of chorea. We thus establish the fact, making large allowance for error (which from the nature of the material cannot be wholly excluded,) that the mortality of children directly from chorea is exceedingly small, yiS A FATAL DISEASE. 75 and that seven is the youngest age at which it is known as a fatal disease in a very extensive review. We find also, as we should expect in view of the comparative commonness of chorea in children, that the proportion dying with chorea but not of it is greater with them than with adults. But next in regard to sex. In 46 fatal cases (again exclud- ing Dr. Tuckwell's) 34 are females and 12 males. Of the 34 females 8 only were children, the youngest (mentioned above) seven years old, and one described only as 'a child.' As many as 26 were young women, 11 of whom, there is good evidence to show, died of chorea. Of the 1 2 males 9 were adults and only 3 young boys. These latter are made up as follows : i is in Dr. Hughes's first series (No. 6), i is in Dr. Dickinson's table (No. 14), and died of heart affection 'without return of chorea,' and i is a case of the late Dr. Fuller's (No. 9, Case 15, Append. A.) that died under my own observation as then Medical Registrar at St. George's Hospital, after five months of almost persistent convul- sion. These three boys were each eleven years old. It does not appear upon the evidence before us that chorea is ever fatal to males at an earlier age than this, nor have we more than one out of the three whose death is immediately attributable to this disorder. The puberty age, then, is the age of fatal chorea. If we take forty-six as representing the total number of persons dying in this connection, thirty-five would be at or a little above the age of puberty, and twenty-five of these young women. ^ We are thus led to associate fatal chorea with the disturbing incidents of a critical period of life. And the more so inasmuch as in those in- stances where exciting causes are actually discovered and recorded (but a small proportion, it must be remembered, of the whole number), there is frequent mention of some special excitement of those parts and organs which in the plan of nature are properly over-active at this particular time. Thus Dr. Hughes, in his ' There is but one elderly person on the list, a woman aged fifty four, who had had chorea for four years. — Dr. Dickinson, loc. cit., No. 15. 76 CHOREA second series of cases, finds the genital organs unusually excited in three of the four cases examined, while in Dr. Dickinson's table out of nine dying directly of chorea, and with causes assigned, there are three in whom these causes are distinctly sexual. And not only does fatal chorea attach peculiarly to the emo- tional sex and the emotional time of life, there are but few instances of it out of the few fully detailed cases which constitute our entire material in this matter where mental excitement does not concur with the bodily over-movement. Such mental dis- turbance embraces all varieties, from the hysterical to the maniacal. Here again, therefore, a distinction appears between the fatal chorea of adolescence and the non-fatal chorea of child- hood. The latter, even in its greatest violence, is commonly quite apart from emotion. The disorder acquires this mental admix- ture at the same time that it reaches what may be called its dangerous age. It will be perhaps better in this place to cite instances rather than to quote figures. The condition to which I am alluding is manifested by particular incidents. Thus in a case related by Dr. Hughes (first series, loc. cit. p. 390), a man of twenty-five, with violent and ultimately fatal chorea, was ' sensibly hurt and shed tears when the bystanders observed that he could restrain the movements if he pleased.' In a second case, by the same author (second series, p. 250), that of a girl of eighteen, the symptoms ' which were at first slight were suddenly aggravated by fright caused by a patient in the same ward, and she became almost maniacal.' In a third instance (from the same series, p. 251), a girl of sixteen, where ' there was unusual excitement of the generative organs,' the symptoms at first * partook of the character of hysterias, the patient appeared strange in her move- ments, and fancied that others were laughing at her.' In a fourth, a boy of sixteen, ' supposed to be addicted to secret vice, his early movements excited the derision of his comrades. Their conduct depressed him much, and he went suddenly into a fit, after which violent and universal chorea supervened.' In all three of Dr. AS A FATAL DISEASE. 77 Peacock's fatal cases mental excitement is a prominent feature. In one of the very few cases that are recorded of chorea fatal as early as ten (Dr. Dickinson, loc. cit. p. 2), it is related of the child (and some will probably regard the relation as trivial), that a little friend of hers had lately died of chorea, she had watched and imitated the movements, and when she recognised them in her own person made sure that she should meet the same fate. If it be remembered from how small a number of examples these instances are taken, even these few quotations will be allowed to have weight in showing the common habit of fatal chorea. I think it may be said, indeed, not only of fatal chorea but of chorea that at any part of its course threatens to be fatal (a comparatively large number), that amongst the symptoms that give alarm nervous ex- altation (so to speak) is generally one. But while the emotional origin of chorea is thus apparent, as well as the direct influence of mental causes in determining its fatal issue, it is not to be doubted that fatal chorea arises in some instances in immediate connection with acute rheumatism. The evidence upon this point now before us is to the following effect : — Out of thirty-two fatal cases dying with chorea, and reported with sufficient fulness to be available (viz., seven of Dr. Hughes', second series, twenty-two of Dr. Dickinson's, and three reported by Dr. Peacock), seven had a rheumatic origin. Selecting out of the thirty-two eighteen who died directly of chorea (viz. , five of Dr. Hughes', ten of Dr. Dickinson's, and all three of Dr. Pea- cock's), we get three — a male and two females, all adults — where the fatal disorder was of rheumatic origin. These numbers corro- borate the conclusion arrived at upon independent grounds (Chap. II. p. 41) — viz., that acute rheumatism, although having but a small share in chorea, is nevertheless veritably associated with it, and especially with its fatal form at the puberty age. Coming now to the morbid associations of chorea in so, far as they are revealed post mortem, the evidence before us fully bears out the belief expressed by the late Dr. Kirkes in 1863, 'that 78 CHOREA further experience would still more positively demonstrate that an affection of the left valves of the heart with the presence of granular vegetations upon them is an almost invariable attendant upon chorea, under whatever circumstances the chorea may be developed.' The singular accuracy of this description, the need of 'almost,' and the justice of applying the observation to chorea of whatever origin, and not limiting it (as some would do even now), to rheumatic chorea, or even to chorea fatal as such, will be seen in what is now to be said. Out of all the cases in which the condition in question is especially mentioned I can find but five where the heart valves and pericardium are reported healthy, one of these being an elderly woman. Wherever the heart is affected the mitral valve is affected ; the aortic valves are implicated often, the pericardium sometimes. This heart affection occurs equally in those that die in connection with chorea, and in those that die directly of it. Thus, of Dr. Dickinson's twenty-two there are, as I reckon, twelve dying with^ but not of^ chorea ; of these ten have mitral valve vegetations, and two have not. Of the ten dying of chorea seven have vegetations, and three have not. In Dr. Peacock's three cases, all dying of chorea, two have vegetations, and one has not. The valve deposit, which for short- ness I thus name, may be either old or new, and variously con- nected with the mitral valves on their auricular side. The condition would seem to be an accident of the disease it accompanies, having but little apparent connection with its fatal result. It so happens that of the five cases just mentioned as being the only examples of chorea occurring with perfectly healthy heart, all save one, the old woman, are typical instances of deaths in and by chorea. Three were girls at about the puberty age (Nos. i, 5, and 8 in Dr. Dickinson's table), and one is a girl of Dr. Peacock's, aged twelve (St. Thomas's Reports, vol. viii. p. 29). With the exception of this observation in regard to the heart and pericardium, there is little to be gathered in the material now before us as to the morbid anatomy of fatal chorea. I need not AS A FATAL DISEASE. 79 again refer to Dr. Dickinson's investigations as to the minute nervous changes in seven of the cases here mentioned, nor to those of Dr. Tuckwell upon embolic plugging, in the paper above quoted. Speaking generally, it must be said that the state of the brain and cord is not described in sufficient detail to be instructive. In a large proportion the brain, in a few the cord, is described as 'congested.' In the case of the boy to whom I have alluded as the single example of the kind in my own experience or in these tables, it was observed that ' the grey matter of the cord was altered and yellow in patches,' but there was no minute examination. How far the foregoing particulars may serve to fortify or dis- turb pathological doctrines I do not now inquire. Their practical teaching is obvious. We are justified upon this evidence in look- ing upon severe chorea at the puberty age, especially in connec- tion with sexual excitement or with acute rheumatism, as a dangerous disorder apt to terminate fatally. It is true that we do not number many fatal cases, but it is also true that we do not meet with many, whether fatal or otherwise. The condition of such patients is the more precarious when, as is commonly the case, there is emotional as well as motor disorder. Such nervous exaltation, which is commonly altogether absent in the more familiar chorea of childhood, is with these both the sign and the source of danger. They are in a state of extreme sensitiveness, and lay hold of trivial circumstances to aggravate their disquiet. The smallest incident, the conduct of bystanders, undeserved reproach or ridicule, or even the sight of a patient affected *like themselves, will often have its immediate prejudicial influence in a degree which those who have little experience of this kind of convulsion (whose actual title to the name of chorea, as Dr. Bright's cases show, is often far from obvious), will find it difficult to believe. The question cannot but suggest itself, whether in these circumstances we address our treatment suffi- ciently to this mental state. But the facts before us have this further teaching. If we 8o CHOREA exclude puberty, chorea of whatever violence is hardly dangerous to life. If we exclude both puberty and the female sex, chorea of whatever violence is not dangerous at all. Now violent chorea is by no means uncommon with boys. Thousands of examples of it must be comprehended within the period we are now review- ing. And yet in all that time, and from so many fields of obser- vation, we get but one boy, aged eleven, dying of chronic chorea after five months, and probably with sclerosis of the cord. Chorea therefore, we may say, extremely rarely fatal in young girls, is practically speaking, never fatal in young boys. If we add to this, what will not be denied, that permanent disablement from chorea is very uncommon, and that there is no treatment that even pre- tends to avert such after-effects, we get a strong argument in favour of leaving childish chorea alone. It will be further strengthened by a perusal of the valuable papers, published in 'The Lancet' of November i8, 1876, by Drs. Tuckwell and Gray, upon ' The Expectant Treatment of Chorea,' The conclusions ^ to be derived from the foregoing review may be expressed as follows : — 1. Chorea, regarded as a disease of itself fatal, belongs almost exclusively to puberty, and especially to female puberty ; its im- mediate exciting cause having distinct reference, in many instances, to conditions of unusual sexual excitement 2. Besides the operation of sexual causes mental disturbance has to be reckoned ; not fright only, but worry, anxiety, and despondency also, while the force and influence of such impressions is to be seen in the course as well as in the origin of fatal chorea. 3. Acute rheumatism appears as a cause of fatal chorea in but a small proportion of cases ; yet the association, infrequent as it is, is distinct and unquestionable. 4. Chorea in its fatal, no less than in its non-fatal forms, shows 1 I had intended to supply an Appendix to this Chapter, setting forth in detail the figures upon which the main conclusions are based, but have refrained from doing so because the material in that shape would occupy much space. The de- ductions here insisted on stand out so clearly that the more general statement of the text will probably be thought sufficient. AS A FATAL DISEASE. 8i strong preference for the female sex at all ages. Children, how- ever, very rarely die of it, and boys, practically speaking, never. 5. Mental excitement, in varying degree (although not amongst the symptoms of ordinary chorea), is met with in so large a pro- portion of its fatal examples that we are justified in regarding this concurrence as of bad augury. 6. ' Vegetations,' new or old, on the auricular surface of the mitral valves, with or without similar deposit on the aortic valves, and sometimes with pericarditis, are met with in the great majority of cases dying of, or with, or shortly after, chorea. This condition, however, does not, as a rule, contribute directly to the fatal issue ; it is found equally amongst those that die with and those that die of chorea ; and in some of the most marked and typical cases of fatal chorea the valves of the heart have been found absolutely healthy. 7. There is no other post mortem condition, except that which concerns the heart, occurring with sufficient frequency or unifor- mity to be regarded as characteristic of fatal chorea. 82 CHOREA. CHAPTER V. THE PATHOLOGIES OF CHOREA. Connection with rheumatism — with spinal meningitis — with pericarditis — with enfeebled nutrition — The embolic theory of chorea — Vascular and nervous changes — Disturbed nutrition of cerebral ganglia — Altered states of blood — Chorea a functional disturbance — Continental opinion — Summary. I PROPOSE to set down in this place, in something like chrono- logical order, a short summary of the several hypotheses as to the pathology of chorea which have been put forward since the time that the disease was first accurately described. The connection of rheumatism with chorea was first asserted at the beginning of this century. Dr. Bright mentions that in the * Syllabus of the Practice of Medicine,' published at Guy's Hos- pital in 1802, 'rheumatism is distinctly mentioned as one of the existing causes of chorea.' He adds that in later editions of the same work, as in that of 1820, chorea is said to alternate with acute rheumatism, 'but through what organ or by what interven- tion it occurs is not conjectured.' In the following year, and independently, as he expressly asserts, of these earlier observa- tions, Dr. Copland drew attention to the rheumatic origin of chorea, upon the evidence of a case which he quoted of rheumatic pericarditis and disease of the membranes of the spine, with which chorea alternated. This change from rheumatism to chorea he sought to explain by ' metastasis from the joints to the membranes of the spinal cord.' 'In nearly all the cases,' he writes, 'there has been a marked disposition of the rheumatic inflammation to PATHOLOGIES. 83 recede from the joints and attack the internal fibro-serous mem- branes, as those of the cerebro-spinal axis and the pericardium.' ^ A more precise reference to the pericardium in association with chorea occurs in the paper by Dr. Bright, from which I have just quoted, on ' Cases of Spasmodic Disease accompanying Affec- tions of the Pericardium' (Med.-Chir. Trans., vol. xxii.). In this communication the author dissents from the conclusions of Dr. Copland, connecting chorea with spinal meningitis, and expresses his belief that a much more frequent cause of chorea is pericarditis, ' although in some instances the coverings of the cerebro-spinal mass might be implicated. ' From the inflamed pericardium, as Dr. Bright supposed, 'an irritation was communicated to the spinal cord,' of which irritation convulsive movement was the consequence. It is to be remarked, however, that Dr. Bright's cases, as des- cribed by himself, are illustrations of spasmodic disease rather than of chorea. In one of them there were epileptic seizures and tetanic spasm \ in another the spasms put on the ' character of most vio- lent convulsions,' 'more violent,' it is added, 'than is almost ever seen in chorea.' Both these cases were fatal. In some of the other examples 'there might be doubt,' as Dr. Bright admits, 'of the correctness of the diagnosis.' What this paper seeks to make out is indicated by its title — the connection, namely, between vio- lent and often fatal muscular spasm and pericarditis, the patients not being children, but young men and women. In order to show how little such views found acceptance in this country, and how vague and indefinite was the pathology of chorea more than ten years later, Dr. Todd's opinion may be next quoted. 'It is easier,' says this physician (in lectures published in 1854, but delivered some time before), ' to say what chorea is not, than to describe what its essential nature is. We may regard it as a disease which depends upon a debilitated state of the system, which does not in any way arise from an inflammatory or hyper- semic state of any part of the great nervous centres or of other ^ See Copland's Z??V/zo«ar)/, art. 'Chorea.' G 2 84 CHOREA. organs. Almost without exception, in those cases which terminate fatally, we fail to detect any morbid alteration which physiologi- cally could give rise to the phenomena.' ' If I were to refer to any particular part, it would be to that which may be regarded as the centre of emotion. The remarkable frequency with which the attack has been traceable to fright as its cause points clearly to this part of the brain as the priijiuin movens in the production of choreic convulsions. The chain of phenomena would then be as follows : first, a peculiar diathesis ; secondly, a more or less en- feebled nutrition ; thirdly, a strong mental impression which dis- turbs the centre of emotion, and through it deranges the action, more or less, of the nervous system and of a corresponding portion of the muscular system.' ' It was eleven years after Dr. Bright's paper, namely, in 1852, when the reality of the connection between rheumatism and chorea had been recognised by text books as well as the occurrence, in the latter affection, of systolic murmur, that Dr. Kirkes wrote his celebrated essay, destined to exercise an important influence upon pathology, ' On some of the principal effects resulting from the detachment of fibrinous deposits from the interior of the heart and their mixture with the circulating blood.' ^ Towards the end of this paper, in the form of an incidental remark, occurs the pas- sage which may fairly be regarded as the origin of what is now called the embolic theory of chorea. ' I would suggest,' writes Dr. Kirkes, ' that many functional disorders of the nervous system, especially chorea, may be thus explained [i.e. by cerebral embolus]. The frequent existence of a cardiac murmur in chorea, and the presence of warty vegetations on the valves of the heart so commonly found in fatal cases of the disease, are in favour of such a view.' Of the numerous modes of application of this re- mark it is impossible to speak in detail. With the knowledge of a frequent concurrence of chorea and valvular murmur, coupled 1 Todd's Clinical Lectures, ' Nervous System,' p. 442. ' Med.-Chir. Trans., vol. xxxv. PATHOLOGIES. 85 with the very general belief in some connection between chorea and rheumatism, it was certain, even if Dr. Kirkes had failed to make this special reference, that the phenomena of embolism would be made use of, sooner or later, on behalf of a disorder sorely in want of explanation. Of the development of this doctrine, and the mode in which it is now expressed, Dr. Hughlings Jackson may be taken as the exponent. 'I suppose,' says this pathologist, 'that the excessive movements occurring either in chorea, or in epilepsy, or epilepti- form seizures, are produced by discharges of grey matter, which, except for great instability from over-nutrition (not better nutri- tion), is healthy. We cannot expect to discover, with our present means of research, the alterations in grey matter on which exces- sive discharges depend.' ' My opinion is,' he goes on, ' that the direct pathological state leading to instability of grey matter, pro- ducing choreal movements, is increased quantity of blood in the periphery of the capillary district embolised.' ' Of course,' he adds, ' this opinion is hypothetical, and so is every one's opinion as to the nature of lesions insusceptible of anatomical demon- stration.' ^ As regards the seat of these lesions, Dr. Hughlings Jackson, following Russell Reynolds, fixes upon the corpus striatum, insisting that there is frequently disease of that part of the brain which superintends the movements of the tongue in uttering syllables. He asserts, moreover, ' the frequent one-sided nature of the movements of the limbs, and their often dying out into definite hemiplegia^ (words which I venture to italicise), 'as pointing to disease at or near the corpus striatum.'^ It has not escaped Dr. Hughlings Jackson that the special character of choreic movement as contrasted with the convul- sion of epilepsy needs some apology. ' The elaborateness of the movements,' he says, ' is strong warrant for the inference that the changes causing them must be seated in the brain and not in the ^ British Medical Journal, Dec, 23, 1876. 2 London Hospital Reports, 1864 86 • CHOREA. cord ; and he thinks it ' most probable that the convolutions are the parts diseased.' From views thus mainly hypothetical we may turn to those of Dr. Dickinson, arrived at directly from post-mortem evidence, and which, in the opinion of their opponents, only fail when the attempt is made to apply them to the actual symptoms of the disease in question.^ It is impossible in this summary to re- produce an adequate account of the facts upon which Dr. Dickin- son depends for his conclusions. These facts are based mainly upon a minute examination of seven fatal cases of chorea, or, as I should prefer to describe them, fatal cases with chorea, in three of them at least the choreic movements being of no great severity.^ The morbid changes, described as remarkably constant in kind and place, are thus summed up by the author. '- In place the changes affected both brain and cord. As regards the two sides of the body they were either exactly symmetrical or tended to be so. The parts of the brain most amenable lay between the base and the floor of the lateral ventricles in the track of the middle cerebral arteries ; the substantia perforata, corpora striata, and the beginning of the Sylvian fissures. In kind the changes were all connected with vascular disturbance. The injection was gene- ral to all the vessels, most marked in the arteries ; when the sources of haemorrhage could be determined they were always arterial ; the degenerations were usually periarterial, and the spots of sclerosis similarly placed. The first visible change would seem to be the injection or distension of the arteries, succeeded by 1 Med.-Chir. Trans., vol. lix. p. 15, 'On the Pathology of Chorea.' 2 Thus in Case i, a girl of ten, the child is described as restless and sleepless, her movements much increased on the 21st, on which evening, ' after acute pains in the lumbar region, she somewhat unexpectedly died ; ' there were vegetations on the aortic as well as the mitral valves. In Case 5, a boy of eleven, the use of the limbs and the power of speech were slowly restored, and the boy was sent into the country convalescent. There ' his heart symptoms became more pronounced, without any return of those of chorea." He died of his heart disease. In Case 6, a girl of thirteen, ' the symptoms were not apparently threatening, and the chorea in particular obtamed little attention.' She too died suddenly and had both mitral and aortic disease. — Vide loc. cii., pp. i «t seq. A similar remark will apply to some of the fatal cases of chorea by other authors. See e. g. H. Jones, loc. cit. p. 560. PATHOLOGIES. 87 extrusion of their contents to the irritation and injury of the sur- rounding tissue. ' Of the cord no region was exempt ; but perhaps the cervical and dorsal regions were usually more affected than the lumbar.' Both white and grey ' shared in the vascular distension. This condition, however, was usually most marked in the vessels be- longing to, or in connection with the lateral part of the grey matter about the root of each posterior horn. This was also the chosen situation of the more definite and special changes.' In commenting upon these changes as a whole, the writer observes : * The nature and steps of the morbid process are open to view, hypersemia, exudation, and its consequences, but not so the causes in which the series has taken origin. Arterial repletion seems mainly concerned in the development of the disease.' As regards embolism, the author remarks not only upon the absence of any signs of such impaction, but upon the constancy with which the changes repeated themselves in certain portions, and the equality with which they affected both sides of the body as conclusive objections to that hypothesis. 'The results,' says Dr. Dickinson in conclusion, 'in muscular excitement rather than in paralysis or loss of sensation, may be associated with the character of the lesions, which are points of irritation rather than planes of section, and as such calculated to produce irritation rather than paralytic effects, not so much to cut off, as unnaturally to excite nervous function.' The author alludes to the similarity between the morbid changes of chorea and those of diabetes, and he concludes with a bold attempt to bend clinical observation into some compliance with the demands of morbid anatomy. ' Every period of life has its own regions of nervous susceptibility ; in childhood the motor ; in adolescence the emotional ; in advancing years the mental, and coevally, or nearly so, that part of the nervous mechanism which instigates glycosuria. Much the same mental impression may make a child choreic, a girl hysterical, or a man diabetic. And thus both in S8 CHOREA. external origin and in the nature, though probably not in the site of the organic changes, we see resemblances and alliances be- tween nervous disorders which in their symptoms betray little similarity.' * In noticing these observations of Dr. Dickinson the advocates of embolism maintain that the condition just described is not in- compatible with that theory. ' The local periarterial softenings,' Dr. H. Jackson suggests, 'account for the choreal paresis,' while the cerebral changes described are not very unlike those producible by embola, regard being had to size of arteries plugged.' He admits at the same time that the embolic hypothesis supposes the cord to be unconcerned in the irregular movements of the disease, and that, as a matter of fact, the absence of embolism is much more common than its presence. Dr. Bastian,^ supporting in some respects the conclusion of Dr. Dickinson, and relying with him upon anatomical evidence, agrees with Dr. Hughlings Jackson in attributing chorea to a disturbed nutrition in the corpora striata and adjacent parts. In place of embolism, however, he would put thrombosis. He points out that the production of multiple minute thromboses ' might easily give rise to minute vascular injections,' but is careful to add that ' other hypersemias, more or less secondary in their duration and mode of origin, may easily be confounded post mortem with those which are hypothetically supposed to lie at the root of chorea.' support of this opinion he refers to three cases of his own, where, with hyperaemia of the ganglia at the base of the brain, and especially the corpora striata, occlusions were found in the small vessels of these parts.^ He sums up his view of the etiology and pathology of chorea: 'I look (certain rare cases excepted), to an altered and often anaemic blood state as its predisposing cause in individuals of a certain age and nervous temperament. Secondly, I look to the initiation in such individuals of a disturbed nutrition ' Loc. cit. p. 39. ^ British Medical Journal, Jan. 20, 1877. ^ Ibid. July 13, 1877, P- 38- PATHOLOGIES. 89 in the corpora striata and adjacent parts of the brain, tending to issue and often actually issuing in what may be called a subacute inflammation of these centres, often characterised in part by the production of multiple minute thromboses.' ^ In harmony with these views, so far as regards locating chorea in the corpus striatum and optic thalamus, and in partial agree- ment with them as accepting capillary embolism of these parts and of their vicinity as one of its causes, we may notice next the opinions of Dr. Broadbent which have especial weight owing to their wide acceptance both in England and Germany. In addi- tion to embolism of the regions just named, Dr. Broadbent * would recognise many other sorts of disturbance of nutrition of the cerebral ganglia, ' as, for instance, peripheral influences arrest- ing the reflex process, direct lesions of the ganglia due to fright or to mechanical lesions,' as possible causes of chorea. ' The pro- cesses of chorea are in fact such as weaken the force of the nervous apparatus without destroying its structure. Hence the weakness of the muscular force and diminished sensibility so common in chorea. Hence also its frequent termination in paralysis.' To the condition of system of which chorea is the consequence. Dr. Broadbent gives the name of 'delirium of the sensory motor ganglia of the brain. ' Having thus reached, in our search after the anatomical basis or material of chorea, the point at which it is affirmed that the nervous apparatus is weakened., but not destroyed, and that the condition of the nervous ganglia concerned is best described by such a word as delirium., we may fairly conclude that anatomical hypothesis has pretty well exhausted itself. But when physical changes are lost sight of, there still remains a wide field of conjecture, if not of research. We leave the tissues, and make appeal to the blood. Variously expressed by various authors, and favoured in part 1 British Medical Journal, Jan. 20, 1877. 2 Ibid. April 17-24, 1869. 90 CHOREA. by some of those I have just quoted, the doctrine which connects chorea with an altered condition of the blood finds perhaps the frankest and most unreserved statement at the hands of Dr. John Ogle, with whom Dr. Hammond, of New York, fully agrees.^ It may be objected that the hypothesis does not so much account for the origin of chorea as for its consequence. Recognising the frequent occurrence of fibrinous deposits on the heart's valves in chorea, Dr. Ogle ^ is led to regard these ' as results of some ante- cedent condition of the blood, common also to the choreic condi- tion.' In rheumatism and in anaemia, ' conditions both of them associated with chorea,' Dr. Ogle observes, ' an excess of fibrine in the blood, which renders it very prone to be precipitated.' ' May not this hyperinosis,' he asks, ' be the explanation of the coinci- dence alluded to ? Speculation might suggest that the fibrinous deposits arise from some interference with the degree of solubility of the fibrine induced by the presence of some ununited elements within the blood, produced by the excessive muscular action and other functional disturbance which exists in the choreic state," this being not in any way related to this state as a cause, but as a consequence.' From conjectures like these the transition is easy to a pathology of chorea which makes no attempt at exact anatomical descrip- tion, but refers, in terms which, although general and inexact are yet sanctioned by physiological use, to altered cerebral nutrition and abnormal conditions of nerve discharge. Among the most striking and satisfactory of such hypotheses is that of Dr. Barnes, who has been led to consider chorea in its connection with pregnancy. ' The condition upon which the latent disposition to chorea de- pends,' he says, 'is some change of nutrition or of structure of the nervous centres, unimportant under the ordinary conditions of life, but liable to be called out into renewed activity linder that special increase of central nervous development which is the constant at- * Y\.z.vnTS\Gw<}i on Diseases of Nervous System, p. 614. ' British and Foreign Med.-Chir. Rev.,]aX\. 1868. PATHOLOGIES. 91 tendant upon pregnancy.' ^ The chorea of puerperal women Dr. Barnes believes to be almost always a revival of the same disease in childhood, having pregnancy for its immediate exciting cause, in virtue of ' the exalted central nervous irritability ' of that condition, which is thus ' a test of the soundness of the nervous system.' As nearly in accord with these views, I may allude next to the opinions of Dr. Handfield Jones ; and here, even more than in the previous instances, comes the difficulty of doing justice to the author in such curtailed statement as the present design necessi- tates. The following quotation, however, gives in Dr. Jones's own words a summary of his opinion. For its illustrations I must refer to his well-known work.^ * The view which I take of the pathological events in chorea is the following. The motor centres especially, and also not unfrequently the intellectual, emotional, and sensory, in persons of weak organisation, fall into a state of paresis, either in conse- quence of a shock or more gradual injury, or of some toxic matter in the blood, or of peripheral irritation, all of which may generate the same peculiar condition. The paresis in all these parts may take the form of hyper-excitability or of paralysis, the former being- much the more frequent. The nerve exhaustion, aggravated more or less by the jactitation, involves the vaso-motor nerves of the cerebral and spinal arteries especially, and conditionates relaxation of their muscular walls as well as impairment of the tone of the capillaries and haemorrhage. These, however, are of course not necessary events, even in fatal cases. Occasionally actual inflam- mation of the nerve-centres results from the hyperaemia. Pulmonary congestion and consolidation may be produced in the same way, and possibly, in some instances, valvular lesions of the heart. This view, that chorea is essentially a functional disorder of the motor centres, is in harmony with its frequency in children and females, whose motor apparatus is more prone to be weak and 1 British Medical Journal, Dec. 9, 1876. 2 Functional Nervous Disorders, p. 361 et seq. 92 CHOREA. irritable, with its affinities to epilepsy, hysteria, paralysis, and insanity.' As for the connection between chorea and rheumatism, it seems to Dr. H. Jones to be best explained by regarding the motor disorder in just the same light as we do delirium, to which it has much affinity. ' In typhoid we have an intestinal lesion, and commonly delirium ; but we do not assume a connection between these, but consider the specific poison to give rise to both. In rheumatic fever we have also often a lesion (cardiac) and delirium, both as co-products of the cause of rheumatism, not one as the cause of the other. If we substitute motor disorder for intellectual, why need we change our view ? ' In this brief statement it still remains to notice some of the theories and researches of continental pathologists. Yet any de- tailed account of these may be excused after the admission of Ziemssen, that 'the sum of knowledge of the pathologico- anatomical changes of chorea is so small that it is scarcely of consequence to attempt to explain it." It is admitted that England and France have been the chief contributors both to the statistics and pathology of the affection, while in Germany the observations of English pathologists upon the embolic origin of chorea and its connection with endocarditis have attracted more attention than any investigations of their own. It is due, how- ever, to the high authority of certain continental observers now to be named that their contributions to this subject should shortly be referred to. Rokitansky, in 1857, asserted the occurrence in chorea of interstitial connective-tissue growth in the central nervous system ; and Steiner, ten years later, confirmed the observation in a single case, and so far as it concerned the spinal cord. He found also, in two cases, serous effusion within the spinal canal, and in one, hcemorrhage at the upper part of the cord at the exit of the nerves. Similar changes, so far as the cord is concerned, together with ' Ziemssen, loc. cit. p. 460. PATHOLOGIES. 93 hypersemia of the medulla and spots of softenings, have been now repeatedly met with. Yet it is to the brain, rather than the cord, that continental observers, upon considerations of physiological necessity, have chiefly looked to discover the structural basis of chorea.^ The cerebral changes, it is asserted, affect the brain cortex and grey substance of the great basic ganglia ; but their precise character is far from being exactly stated, and is made to rest rather upon clinical facts than direct observation. As with the cord, the brain changes are said to include interstitial hyper- plasia, with regressive metamorphosis of nerve elements, some- times confined to one hemisphere or its great ganglia, and some- times diffused over the whole brain, preferring the grey substance, and extending always to the cord, and even the peripheral nervous system. 2 Again, Charcot has recorded some observations upon cases of hemichorea after hemiplegia, in which cicatrices were found at the posterior end of the optic thalamus, and (in two cases) of the anterior corpus quadrigeminum of the affected side. In other instances, where hemiplegia followed chorea, the same observer found a blood clot as large as a nut in the posterior half of the optic thalamus. In accepting the evidence of such cases, however, we are clearly transgressing the proper limits of our subject, and complicating the pathology of chorea with that of paralysis. A similar objection applies to many of the other quoted cases, where it will be found that the chorea is combined with some other affection, as with mania or encephalitis. It is admitted, indeed, by Ziemssen and others — and I shall presently quote instances of this kind — that ' chorea may occur as a transitory phenomenon in * Ziemssen. Cycl. , loc. cit. 457 et seq. * 2 As regards the latter, the observations of Elischer may be quoted. He found the changes just described for the cord equally marked in the peripheral nerves, the connective tissue between nerve bundles being greatly developed, and the medullary sheaths of the nerve fibres tumefied, while their axis cylinders had dis- appeared. The nerves examined (the median and sciatic), seemed to the naked eye abnormally small and flat. 94 CHOREA. the course of cerebral lesion, and presently give way to other and severer disturbances.' * It is misleading to adduce the post- mortem appearances in such cases by way of evidence of the changes attending chorea. Lastly, experiments have been made upon animals by Chau- veau and others,^ and it has been shown that the muscular twitchings of dogs persist after section of the cervical cord, and, further, that these may be produced or increased by mechani- cal irritation of the posterior columns. Thence it is concluded that the limb-twitching depends on the cord, and not on the brain. But such information is not in point, for the simple reason that this canine affection is not really chorea, or at all like it. In reviewing the several observations and conclusions which have now been enumerated, it is easy to see that the theories in reference to chorea which have successively gained acceptance represent so many attempts to accommodate this particular affection with the pathological doctrines of their day. When first emerging from the obscurity of humoral pathology, chorea, in virtue probably of the pain that often attends it, is found in some sort of alliance with rheumatism. Presently, upon the observation of its occasional association with acute rheumatic arthritis, the prevalent belief in metastasis is appealed to, and the muscular disorder is accounted for by an inflammation transferred from the joints to the spinal cord. Next, and following the researches of Marshall Hall upon the functions of the cord, we find the prin- ciple of reflex irritation made use of to explain the dependence of chorea upon pericarditis. At a still later period, and when further observations of the heart symptoms of chorea had made the appli- cation easy, comes the discovery of embolism, both to explain the cardiac disturbance and to satisfy those phenomena of the disease which better study had shown to depend upon the brain rather than the spinal cord. ' See for example Case 4 in Appendix. 2 Arch. -Gen. de Mdd., March, 1866, 455. PATHOLOGIES. 95 But while the pathological history of chorea has been one of revolution, its clinical history (in so far as the two may be separated) has been one of almost continuous progress ; and it has come to pass with the gradual accumulation of facts, that, while precise and definite hypotheses have been found faulty and insufficient, the only adequate pathology of chorea hardly goes further than to translate its symptoms into physiological language. We have mere definitions of the disorder which are unassailable, which venture nothing and explain nothing ; but what is wanted is some explicit statement of the anatomical conditions on which chorea depends which may be brought into harmony with its actual phenomena. 96 CHOREA. CHAPTER VI. THE PATHOLOGIES OF CHOREA IN THEIR APPLICATION. Clinical conditions to be satisfied — The symptoms of embolism compared with those of chorea — The direct evidence in favour of the embolic theory — Perivas- cular degeneration of the brain and cord as a cause of chorea — Hypothetical explanations — Chorea ascribed to temporary trophic changes — AppUcation of such theories — The ataxy of chorea. I TURN then once more to chorea itself, and would place side by side with it the several hypotheses which have just been enumer- ated. No theory can be finally accepted which fails to satisfy all the conditions of the problem. There are certain features of chorea which are obvious and beyond dispute, as that it is a child's disease, that it prefers the female sex, that it is apt to recur, that it affects the arms, hands, and face more often than the legs, that it is more often both sided than one-sided, that complete recovery is the common rule, and death or any permanent disablement the rare exception and so forth. Whether, therefore, it be alleged that embolism, or thrombosis, or perivascular softening, or * disturbance ' in some motor centres, or ' toxic influence ' is at the root of chorea, each of these conditions needs for its justification to be brought into relation and harmony with the known symptoms of the dis- order it professes to explain. Now, if this comparison be fairly made I think it will appear : — I St. That there is no morbid condition as yet anatomically described which, so far as we have independent knowledge of it, is capable of producing symptoms at all similar to those of chorea. 2nd. That the hypotheses which, apart from demonstration, really provides the best for the actual phenomena of chorea, do APPLICATION OF PATHOLOGIES. 97 not in fact present any distinct picture of a definite morbid change. 3rd. That having regard to the special characters of chorea, it is difficult to conceive of any morbid condition whatever, anato- mically demonstrable, calculated to produce it, or indeed com- patible with it. I. The morbid anatomy of chorea, or as much of it as has survived, begins, as we have seen, with its association with endo- carditis,^ an association soon to be supplemented and explained by the observations of Dr. Kirkes upon embolism in 1852. Taking, therefore, this, the most concise and ingenious of all the theories having a purely anatomical basis, we may inquire, in the first place, whether embolism, so far as we have independent knowledge of it, is capable of producing symptoms at all similar to those of chorea. Now, the ordinary symptoms and course and cause of embolic obstruction are known. Making allowance for great diversity in the response to cerebral injury, as well as for modifications due to the extent, size, and site of the plugging, our actual information under these headings may be summed up generally as follows : — {a) For sympto7ns : a sudden vertigo or an acute head pain, with faintness or sickness ; a more or less complete hemiplegia ; im- paired intelligence and sensation ; and, if the hemiplegia be right- sided {as it most often ts\ either aphasia or inability to utter articulate sounds, (d) For course : a gradual but interrupted decline, or an incomplete and precarious recovery ; nutrition 1 In speaking of the several anatomical conditions which have been definitely described as representing the material expression of chorea, it is unnecessary to refer again to doctrines which are now abandoned. Yet the observations of Dr. Bright in 1841 upon the connection between pericarditis and chorea have this singular value ; they point to a connection which, although different in form from that which Dr. Bright asserts, is really implied in the evidence which his paper affords. There is reason to believe, as we shall presently see, that severe and fatal choreic convulsion is sometimes in direct relationship with endocarditis. It is in the highest degree probable that Dr. Bright's cases, although referring expressly to pericarditis, are in fact illustrations of this connection, inasmuch as the endocar- dium and the pericardium commonly suffer inflammation together. H 98 CHOREA. changes in the muscles of the affected side ; a special liability to recurrence of the first fit ; often a fatal termination by way of coma. Such or such like clinical features of embolic cerebral obstruction are accounted for by reference to its morbid anatomy. Its usual seat, mainly determined by the distribution of the middle cerebral artery, is the left rather than the right side of the brain, while secondary softening is an early consequence of the original accident, {c) For cause : the conditions which belong tD degeneration ; to endocarditis ; to acute rheumatism ; and to the ]:)uerperal state. In other words conditions belonging to advanced life, or to the time between puberty and middle age. Such, I say, is the general description of cerebral plugging, so far as it has been observed clinically and anatomically ; and if the account have reference to minute embolism, we have but to add that there is often active delirium, high temperature, and a train of symptoms, rapidly fatal, which resemble most specific fever.* How far does all this apply to chorea ; to a disease confined almost to childhood; favouring the female sex ; preferring the left side rather than the right ; seldom sudden in its access ; hardly ever exhibiting aphasia, high temperature, vertigo or sickness ; jjroducing no special wasting of the affected muscles ; implying little danger to life or limb, and apt to disappear at that precise period of life when, with the greater frequency of endocarditis and the setting in of degenerative changes, the liability to embolism and thrombosis is largely increased ? Putting together all that we know of the plugging of cerebral vessels, large or small, in its symptoms, associations and subjects, it is directly opposed to all that we know in respect of chorea. ^ If it could be shown that this particular morbid condition was constantly present in chorea, ' See, for example, two cases of cerebral embolism reported by Dr. Dickinson, Britixh Medical Journal, May 21, 1881, p. 795. - The connection of chorea with acute rheumatism and with child-bearing may be alleged to the contrary. But the rheumatic connection, as I have endea- voured to show elsewhere, is rare, and in its nature peculiar ; while the chorea of child-bearing, as Dr. Barnes has pointed out, is almost always but the revival of a former chorea in childhood. APPLICATION OF PATHOLOGIES, 99 the observation would offer a new difficulty ; we should still have to look elsewhere for the structural basis of the disorder. But we have still to inquire upon what ground of actual obser- vation this doctrine of vascular plugging in chorea depends. ' I am aware,' says a very candid and fair advocate' of this belief, ' that the morbid anatomy of chorea is not considered to lend much support to the attractive hypothesis of embolism.' Drs. Wilks and Moxon have never met with microscopic emboli in any instance. In one case where, together with chorea, hemiplegia had followed embolism, ' there were no discoverable emboli in the small vessels.' These authors are unable, indeed, to recognise any constant morbid appearance after death in chorea. Similarly Dr. Gowers and Dr. Ferrier have failed to discover emboli after diligent search. It is not too much to say that instances of minute embolism in association with chorea are conspicuous by their rarity. Cases of the kind, as those of Dr. Broadbent and of Dr. Tuckwell,^ are quoted over and over again, but it is admitted that they conflict with the general experience. ' I have never seen an instance,' says Dr. Dickinson, ' in which the well-known blocking [of embolism] as found after death, has been conjoined in life with choreic symptoms ' {loc. cit. p. 36). If this theory of vascular plugging is to be established, it must be, as Dr. S. Mackenzie admits, 'upon evidence of a clinical and physio- logical nature.' Where is such evidence to be found ? It would appear, on the contrary, that the condition of minute vascular plugging, whether of embolism or thrombosis, is both insufficient theoretically, so far as our knowledge goes, to account for the special phenomena of chorea and, as a matter of fact, only excep- tionally met with in connection with it. The same may be said in part of the nervous changes pointed out by Dr. Dickinson, and shortly described in the last chapter. 1 Dr. S. Mackenzie, British Medical Journal, Dec. 23, 1876. 2 Medical Times and Gazette, 1875, P- 4^2 ; British and Foreign ^edico- Chirurgical Review, 1867 ; St. Bartholomew's Hospital Reports, vol. v. H 2 lOO CHOREA. Although this condition, consisting in spots of perivascular de- generation distributed symmetrically throughout the brain and cord, was found wherever it was looked for (not only in chorea, but in diabetes as well), it is obviously not the kind of change we are seeking, and, being found, it is impossible, without a revolu- tion in physiolog}', to reconcile with it the actual phenomena of chorea. How can these degenerated spots, symmetrically arranged, affecting the cord as well as the brain, and tending to ultimate ^ dubious grounds.] Case XL — Left Hemichorea implicating lower part of left face, left hand, and left foot in a young pregnant wo?nan newly married, a month after rheumatic attack. Jane C, aged 21, ironer, four months married, and in third month of pregnancy. Two and a half months before admission she had had pains, swelling and tenderness in the small joints of both hands and the left foot, for which she kept bed a week. Five weeks before ad- mission {i.e. something over a month from supposed rheumatism and when in good health) suddenly, and from no apparent cause, she felt a twitching or turning outwards of the left hand, the left foot soon par- ticipated, and the left corner of the mouth had downward twitch. These movements were very perceptible on admission, although not violent. The woman had never had chorea, and, except for headache and neuralgia, knew of no previous illness. There was no rheumatism or neurosis in her family history. A variable systolic bruit was audible between apex and base of the heart, sometimes loud, some- times quite lost. The patient improved in a few days, and left the hospital after a fortnight's residence, nearly steady. [Here was a strictly one-sided affection, involving both face and hand, and foot, in just such manner as irritation of a motor centre would, and without the slightest participation of the right side. If hemichorea at all, then a very perfect and complete hemichorea, i6S CHOREA. pointing to a definite seat of irritation. Yet it was not a continuous restless movement, but an occasional twitching, without muscular in- co-ordination. If the term chorea is to be restricted to the cha- racteristic form of the child's affection, this case would not be an example of it. Yet in its occurrence during pregnancy, partial control by the will, the state of the heart, and the complete recover)', its near alliance to chorea is obvious ; while the exception which it offers in its mode of distribution over the body to the common rule of that affection is very significant.] Case XII. — Chorea in Middle Life occasioned by mental shock. James S., aged 38, a wire-worker, intelligent and unemotional, with no history of chorea, syphilis, or rheumatism, either personal or in his family. He had enjoyed good health up to three years ago, when he lost a child by death. He then became unsteady in the left arm, and soon in left leg and left side of face. Eighteen months ago, on the death of his wife, the chorea became general. He was for two months in St. Thomas's Hospital, and afterwards at Guy's. In the latter hospital he had an attack of acute illness described as rheumatism, and lasting two months. On recovery the limb move- ments became somewhat less. The patient is in good general health, and least unsteady when employing his hands holding a book or turning over leaves. He is .even able for a while to work at his trade. The choreic affection oc- cupies the limbs, face, and speech muscles. In the limbs it is seen most in movements of extension, giving to his gait as he walks a curious spring for each step. The heart's action is regular except that with some beats there is marked prolongation of the first sound. When lying in bed the movements are almost confined to the upper limbs. The pulse is 94, temperature habitually subnormal, a little over 97°, rarely reaching 98°. The patient remained in hospital for about two months, and im- proved unquestionably in his command of speech and power of walking. His own estimate, however, as to the measure of his re- covery exceeded that of other observers. His poverty and pre- carious mode of living previous to admission made him so far a favourable subject for treatment, and such progress as he made towards recovery was due no doubt to improved nutrition from better living. As already mentioned, he was always best when his hands ABSTRACTS OF CASES. 169 were employed, and when discharged he beHeved himself well enough to resume his employment. [This case affords an example of the very rare occurrence of chorea to a man of middle life, and not excitable or notably feeble in intellect, from the operations of causes similar to those which pro- duce the disease in childhood. It is the more remarkable as he had not suffered chorea in early life. As regards rheumatism, the patient was certamly without it until after the chorea had become confirmed and general. The exact nature of the attack in Guy's Hospital de- scribed as rheumatism could not be made out owing to the man's infirmity of speech. For the same reason this case cannot be quoted to negative the statement that no grown man of full, mental capacity ever yet suffered chorea. The degree of intelligence through all the difficulties in the way of expression could not here be estimated, and there was no evidence as to his condition in this respect when first attacked. He was not conspicuously emotional, was fairly educated, and fond of reading.] Case XIIL — Severe Chorea with Muscular Paresis ; variable ataxy ; extreme agitation of the hand ; benefit to be derived fro?n mechanical support and muscular exercise. Annie M., aged 12, an intelligent and tractable child, lean but of healthy aspect, was brought to hospital with rather severe chorea of about three weeks' duration. The affection was first noticed from her dropping her glass while drinking. The over-movement was now general, but affected the right side chiefly. Both hands were very unsteady, and the right could not be fairly extended from the wrist, but habitually drooped more or less. She was quite unable to stand, or walk, or move the legs in any desired direction. The girl had never had rheumatism, and the attack (which was the first) was attributed to falling downstairs or, perhaps, to toothache and extraction. There was neither chorea nor rheumatism in the family history. The heart was slightly uneven in action and the first sound prolonged. The choreic movement soon became much worse, so as to require special watching. With the full consent and concurrence of the child means were employed mechanically to restrain the limbs. A sheet wide enough to cover the whole trunk was stretched over her and tied round the bed. This was applied and removed at her pleasure, 170 CHOREA. and by its aid she got sleep and soon improved in steadiness. But now with the lessening of the more active agitation a general mus- cular weakness became apparent. The girl lay low in bed, and the inability to stand was now due rather to want of strength than want of steadiness. The rhythm of the heart had become irregular. There was no appreciable wasting. It now became a nice question whether her condition might be best met by rest in bed or by directing such use and exercise of the muscles as was possible. In the belief that the paresis was parallel to that met with in hysteria, and with a view to test- ing that opinion, the girl was directed to be up for a few hours daily. In the course of a fortnight she was able to walk with some support, dragging the legs somewhat, but showing no misdirection. The face was still unsteady, and, as at the first, words were uttered with difficulty and in jerks ; but the stress of the disorder was now chiefly in the right hand, which was utterly choreic, and unable either to be still or to make any attempt at grasping. In pursuance of the same plan that had succeeded with the general disorder, this hand was now bandaged down on a straight splint, the child concurring in this as readily and indeed eagerly as in the case of the sheet. For the first week of this treatment the hand could be felt with the fingers wriggling about under their bandage. After this the hand became rapidly steadier. She was able first to hold a pencil, and soon to write : the return of nvritiiig power being noted to a day a?td eveti to aft hour, while once regained (as in the case of walking), it was veiy soon regained completely. The girl was in hospital altogether about three months, the first fortnight of that time showing a severe degree of chorea, and the rest of it being occupied with recovery which, although slow on the whole, was rapid and almost sudden in its main incidents. [Besides the points for notice already alluded to in the text of this work, this case may be quoted amongst others to show the benefit of restraint. The mechanical aids to steadiness which were given to this girl, if they did not quicken recovery (and I am not certain that they did), were yet grateful to the patient, and were applied only in so far as they were so. Restraint which is not directed and qualified in this way, restraint which is sternly ordered by the doctor without con- sulting the patient, is more likely to do harm than good, and sometimes does very serious harm. But with the patient's concurrence, and with a clear understanding that it may be used and disused at will, me- chanical restraint may sometimes be made use of, on and off, with ABSTRACTS OF CASES. 171 manifest advantage, even in cases more violent than the present. Another point of interest concerns the treatment of the general paresis of chorea and the need of stimulating the muscles to exertion. The rapid transitions of the disorder, and especially the quick recovery of the use of the fingers shown in the present case, are highly character- istic features.] Case XIV. — Chronic Chorea with Tncoiitinence of Urine., emotional attacks ajid temporary Heart Disturbance. Condition of fatuity succeeding. Improvement with discipli?ie. Samuel S., aged 13, a fresh-coloured healthy schoolboy, of blunt intelligence, had pains in the left thigh ten months before admission, shortly followed by choreic movement of the same side. The present is a second attack of three weeks' duration, affecting the left arm and especially the face (the forehead and eyebrows being in constant movement). There is no apparent cause for this second attack. The case, never violent, was extremely obstinate. It was marked by three notable incidents : incontinence of urine ; emotional or hysterical attacks ; and great variability in the character of the heart's action. The incontinence continued more or less throughout his stay. The emotion was exhibited on the occasion of a sudden death in the ward, when the boy had a fit of crying and sobbing with much increase in the choreic movement. The heart, at first regular and without obvious murmur, became for a while irregular or uneven, and de- veloped a variable murmur which exercise made louder, and was post diastolic in time, and heard best just above and within the apex impulse. After many variations this boy fell into a half fatuous state, where, as will be seen from the description, it was extremely difficult to estimate the precise mental condition. The muscle movements, moderate but general, were greatly aggravated on moving, and he was quite unable to walk or stand. He lay habitually on the back, low in' the bed, constantly grimacing, and passing urine involuntarily. When addressed he would sometimes merely grin, and sometimes make an effort at speech, never getting out more than a word. He took food well, but wasted somewhat. His temperature was habitually sub- normal, between 97° and 98°, pulse very variable, ranging from 96 to below 70. When this boy had been over three months in hospital it occurred 172 CHOREA, to Dr. Donkin (who then had charge of the ward and to whom is due the full credit of this treatment) to make trial of moral persuasions to exertion more forcible than had hitherto been employed. The patient was with some difficulty got out of bed, and unavailing attempts were made to get him to stand with the help of some support. This failing, a faradic current of some strength was applied, much to the patient's dismay. The next day he stood, and from that time made continuous and rapid progress, no further severity of urging being called for. The face unsteadiness in lessened degree persisted, but in other re- spects the boy was almost well three weeks from the commencement of the active treatment. [The lesson of this case, so far as treatment is concerned, speaks for itself. Yet, though it yielded rapidly to moral suasion applied at the right time, it was truly a case of chorea as evidenced by the con- tinual movement of the face no less than of the limbs, by the speech difficulty and the condition of the heart. The variability of the pulse and subnormal temperature were further signs which could not be mistaken. What the treatment accomplished was to break the monotony at the time when the aftection, no longer active, had left a stupid boy without the vigour to bestir himself unless strongly roused. Such treatment is only to be commended for such cases, yet its success in this instance is one of the many illustrations we have had to consider of the points of contact between chorea and hysteria. It need hardly be added that faradism has no specific effect, and that any other application equally disagreeable would be equally effectual.] Case XV. — Chronic Chorea in a boy of eleven^ ultitnately fatal [The following case was under the care of the late Dr. Fuller at St. George's Hospital, in 1864, and reported by myself, then Medical Registrar, the post-mortem examination being performed by Dr. Dickinson, then Curator of the Museum.] Leopold L., aged 1 1, admitted July 13, 1864. The boy had before been an in-patient, and was discharged improved, but on returning home his disorder at once returned. At the time of his second ad- mission it had lasted three months. No cause could be traced. The patient was well nourished, of dark complexion, and not unhealthy aspect. The chorea was general but at the first not extreme. He ABSTRACTS OF CASES. 173 was given iron and sulphate of zinc, the latter up to five grains for a dose, and for a short time improved. This early amendment, how- ever, not continuing, he was ordered strychnia with iron, and morn- ing shower baths. At the end of five weeks from his admission his state had become distressing. He got little sleep, had wasted much, the tongue was frequently bitten, and the skin scratched with the finger nails. The liquor arsenicalis was now given in five minim doses, thrice daily ; many other drugs followed, particulars of which it is unneces- sary to insert, since none exercised the slightest beneficial effect. The symptoms progressed without interruption, the skin was excoriated, the tongue bitten, and bed-sores began to threaten. At this ad- vanced period of the disease it is noted, ' There seems a strange mixture of temper with the disease, rendering it difficult to discri- minate between the two. His most violent paroxysms may be controlled for awhile by speaking sharply to him.' Ten days be- fore his death he was tied down, and, to prevent further injury from his scratching, the hands were secured. So he lay like one possessed, struggling and kicking, the mouth and tongue deeply ulcerated, and the body lacerated and bleeding wherever his fingers could reach it. He died September 17, after over two months' residence, and more than five months' duration of chorea. Post-Morte7?i Examination. ^ The body was much emaciated, and the skin rubbed ofT the prominences of the back ; the hair was worn off the back of the scalp, and under its skin was a collection of pus ; the lips were scored with fissures. The brain^ both grey and white, was injected, the ventricles natural. The cord showed on its surface large vessels distended with blood. On section in various situations, its appearance was not un- natural ; but on closer inspection portions of the grey matter seemed duller and more yellow than natural, particularly towards the upper part. External to the membrane were much distended veins lining the vertebral canal. No microscopic examination was made. The heart at the inner edge of the mitral valve showed a line of soft beads of lymph easily detached. The left ventricle was con- tracted. 1 This case is referred to in Dr. Dickinson's paper, Med. Clin. Trans., vol. lix., and included among the 22 fatal cases he has collected. 174 CHOREA. The kidneys were congested. Other organs natural. [Although incomplete in some respects, I am induced to select this case for quotation in this place, owing to its extreme rarity. Chorea in a chronic form occurring to a boy of this age, and persisting up to the time of death, 710 extra symptoms supervening^ but the disease fnaintaifiing its proper characteristics to the very end^ is, as I think Chapter IV. tends to show, an excessively uncommon event. There was no trace of paralysis, no known rheumatic connection, and no cerebral symptoms beyond those of temper. Compare Case by Dr. Peacock, St. Thomas's Hospital Report":^ vol. viii. p. 29.] APPENDIX B. TABLE A.1 132 CONSECUTIVE CASES OF CHOREA, SHOWING ITS RHEUMATIC CONNECTION, STATE OF HEART, EXCITING CAUSES, LIMBS AFFECTED, &c. 1 In the composition of this table I am indebted to notes furnished me, at the time the patients respectively came under my care, by several successive Registrars of the Hospital for Sick Children, during the five years the work has been proceed- ing. The present Registrar, Dr. Abercrombie, has supplied the largest portion ; Dr. Garlick the next largest. 176 CHOREA. SUB-SERIES I. If previous If Rheumatism If Rheuma- No." Name Age 6 Whooping cough previously tism in family I Charlotte O. Whooping-cough Rheumatism 2 Anne N. II ., 3 Henry R. . 9 4 Albert S. . 7 Whooping-cough 5 Fanny P. . 10 Swelling of wrists 6 Emma R. . 61 Whooping-cough and ankles 7 Ellen C. . 8 ,, No Rheumatism 8 Amy A. 8 II ? Rheumatism 9 Rosa R. 7 10 Arthur W. . 8 1 vj II John W. . 7 — Hi 12 Henry P. . 7 — - '■n 13 Amelia B. . 7 — — 14 Alfred M. . 10 — ''Z* 15 Florence B. 6 16 Louisa S. 9 Whooping-cough Very doubtful '■J 17 Susannah M. 7 , , ? Rheumatism I. 18 George P. . 6 — c 19 Agnes D. 8 Whooping-cough 20 Alice H.^ . II ,, 21 Martha C. . 9 ,, 22 JaneC. 9 — — 23 Sarah C. . 9 Wh oopi n g-cou gh 24 Amy D. 5 ,, 25 Beatrice P. . 6 — — 26 Ruth E. . 4 — — 27 Walter N. . 9i 28 Herbert B. . 7 Whooping-cough 29 Anne O. 10 ,, 30 John B. 8 II 31 Mary II. . 8 ,, 32 Emma K. . 10 ,, 33 Anne F. II 34 Alice D. II Whooping-cough 35 Marv H. . 8 Z^ Alice M, . lO .. 37 Rose G. 9 II 38 Florence E. 9 ,, 39 Charles B. . 9 ? Subacute ' This mark indicates throughout that the point in question is not referred to in il. notes of the case. '^ Right hand choreic constantly against all control. ILLUSTRATIONS. 177 SUB-SERIES I. Heart Condition 2nd or Part of Body 3rd attack where Commenced Supposed Cause Result Remarks Mitral regurg. Twitching face — Well " Rapid Im- >i provement — — No cause, ' gradual, father insane Nearly well — — No cause, emotional WeU Mitral dist. Began in right hand — ,, - Mitral regurg. 2nd — No cause 1 J — Began in hands — II — In left hand — II Syst. Murmur In left side (? hand) No cause i-i_ — — Fright f 1 — Left side 1 J II Murmur Right side No cause Mitral Mur. ,1 After measles )> — 2nd Right side (both attacks) No cause II Mitral regurg. Right side ,, II — Right hand After ' low fever ' 1 1 — Right side Harsh treatment at school Syst. murmur 4th Right arm ? Fright, overtaxed at school 1 1 — — Fright ,, — Left arm Fright at school II — 2nd i f Overwork at school 1 1 Irregular No defined cause ,, Genl. twitching — , , — Right hand Fright J , 2nd Left leg I St attack fright Not improved Regular Left arm & leg None (nervous) Well Both hands Death of brother In statu quo Stupid Fingers & face Fright, sunstroke Well Hands Pinching right hand jj 3rd Left hand & side Left side None 1 1 Right hand Fright Well 2nd — — Improved See 31 Murmur — ? A fall Well Regular Right hand Very studious, and leg ? cause 1 1 — Left hand Worms • 1 Nervous Regular, mur- mur variable Hands No cause 1 1 , 1 • No cause ' denotes none ascertained after full enquiry. N ijS CHOREA. SUB-SERIES I— CO}} tin tied. No. Name Age If previous Whooping-cough If previous Rheumatism If Rheumatism in Family 40 41 John B. Gertrude W. 4 Whooping-cough 42 Emma S. . 8 ,, Ul 43 Jane S. 7 " hi 44 Fanny F. II " 45 Louisa A. . 12 46 47 jMary F. Maria N. . 9 loj Whooping-cough Rheumatic fever a 48 49 John B. Frederick M. 9 10 •• y. 50 Katharine S, 6 ,, ^r Louisa J. . 12 >» 52 Emma R. . II •• SUB-SERIES II. No Age If previous If previous If Rheumatism Whooping cough Rheumatism in Family X Marion R. . 5 Whooping-cough 2 Henry M. . 9 ,, — 3 Jane C. 10 — 4 . Eleanor B. . 5 Whoopi ng-cough ? — 5' Florence E. 9 ,, — 6 Charlotte G. 10 ,, — 7 lilizabeth L. 8 > > — 8 Amelia G. . II »i Father rh. 9 Hannah C. . II , , 10 Richard G. . II II Alice W. . 6 ^ J Father rh. 12 Eliza L. 8 1 ^ 13 Katharine S. 10 , , 14 Ernest James T. . 8i ,, 15 Christina A. 10 — — 16 Elizabeth F, 8 Rheumatism 17 Elizabeth P. TO ' 18 Henry M. . 8 Whooping-cough 1 Habitual flexure of right wrist developed ; said to have been palsied ou that side ; no distmct account. ILL US TEA TIONS 179 SUB-SERIES I — contimied. Heart Condition 2nd or 3rd attack Part of Body where Commenced Supposed Cause Result Remarks Regular Hands No cause Fright In statu quo Well Nervous ,, Legs and arms No cause ,, Nervous Uneven systolic Twitching arms Stupid apex murmur for 3 years 1 ) " Iridectomy four Twitchinc of years ago, twitch- Regular eyes and face Fright 1 71 statzc quo ing and awkward movements ever smce , — No cause Well A stupid child 2nd — >' .. " 2nd Hands " " Mitral murmur — Fright Improved Regular — No cause Well J, Hands Overwork at school A clever child Hands and legs No cause SUB-SERIES II. Heart Condition 2nd or Part of Body 3rd where Supposed Cause Result Remarks attack Commenced Regular Left arm & leg Well Mental defect ? Right side A fright 2nd — — — - — 3rd — Fright See No. 38 2nd Limbs and face No cause ,, ? Fright Syst. murmur Left side Being run over {i.e. fright) — Limbs gene- rally affected Over study Regular 2nd Right side No cause ? Fright — No cause It 2nd Facial & general ? a fall 'Brain fever' (3atks.) See No. 50 Irregular — Over study Taken out by friends Syst. murmur Right hand Rheumatism Well Regular Rt. hand & arm Over fatigue Improved " 2nd — No cause Well N 2 I So CHOREA. SUB-SERIES W— continued. No." Name Age If previous Whooping-cough If previous Rheumatism If Rheumatism in Family 19 Henry B. . II Whooping-cough 20 21 22 Annie E. Edith T. . Helen K. . II 9 9 " Rheumatism Father 23 Arthur W. . 10 ,. 24 Edward F. . 7 ,, Rheumatism Mother 25 Alfred G. . 7 >, — — 26 Sarah B. . 9 ,, Sister 27 Arthur C. . II ,, Rheumatic fever 28 29 30 Rose L. Louisa J. . Anne G. 7 9 2 Whooping-cough Rh. Just before 31 Hannah B. . 9 ,, Rheumatism 32 33 34 35 1 36 37 Sydney P. . Annie P. Albert H. . Anne P. Mary Anne D. . Elizabeth H. 6 9 II 3 9 11 Whooping-cough Redness or swell- ing, feet & hands Mother & father Mother's family 38 Annie G. 10 >> Rheumatism ^ 39 Margaret M. 7 .. 40 Annie C. 10 '• 41 Lydia H. . 7 42 43 Eleanor W. James F. 6 8 Whooping-cough 44 Olive T. 4 Rheumatic fever Family 45 46 47 43 Florence F. Edward S. . William B. . Elizabeth R. 8 9 6 9 Whooping-cough Rheumatism Pain in joints, no swelling or redness 49 Ellen B. . 10 .. 50 Jane L. W. 7 ? Whooping-cough Father rh. Sister SI Horonce M. 7 Whooping-cough ^ Hands and knees swollen 14 days, but did not keep bed. ILL USTRA TIONS. i8i SUB-SERIES \l— continued. {Heart Condition 2nd or 3rd attack Part of Body where Commenced Supposed Cause Result Remarks Syst. murmur (at first) 2nd — Frightened by horse Well Regular 1 1 General Hands No cause Over study No cause No cause. In ist > > Mitral regurg. 2nd General attack fright, dog, on side affected »> Irregular — — ... Developed chorea on taking Aortic regurg. ■~- •^ cold at Cromwell House ; was taken out by friends Mitral 2nd Legs Right hand No cause Fright, horse run- Well ' ' ning away with him J i * Regular Left hand Left side No cause 1 i »> General ? Rheumatic fever Falling down and breaking a jug Arms No cause i» [[ 2nd Left side Fright Improved ,, Left hand No cause Well Syst. murmur Right arm & leg ? Rheumatism » » Regular General Fright 1 ,, Mitral murmur Right hand No cause ? Rheumatism, has » » Regular 2nd Left arm (not complained of pain (uneven) leg) in left side since so- called rheumatism >> Mitral 2nd Left side General (began in right hand) Fright (both attacks) >> Begun with lan- Uneven ? >> guor and extreme nervousness Regular 3rd General (began in right arm) Fright, dog jump- ing up right side Improved Syst, murmur General No cause Well Regular 2nd Hands »» >) Syst. murmur General ? Rheumatism >> Appearance of mere shyness Not regular ,, Fright Improved Regular Left hand ) > WeU Aortic obstn. Right hand No cause 11 Mitral murmur 2nd Left leg >» ist sound thick at apex 2nd Right side Fright (on right side) II Not quite reg. 3rd Left side most After fall — ist at- tack fright Contending for ) 1 Mitral murmur Left arm & face school prize, much excited thereby Improved ^ Ascertained at Highgate, 1 82 CHOREA. SUB-SERIES III. Age If previous If previous If Rheumatism No. Name Whooping-cough Rheumatism in family I J ane B. II Whooping-cough Rheumatic pains Father 2 Madeline P. II — 3 Emily W. . 6 Whooping-cough 4 Florence P. II — — 5 Alice J. II Whoopin g-cough 6 Elizabeth S. 7 Rheumatic pains Father 7 John B. II Whooping-cough Pains Mother 8 Rosa R. 10 ,, 9 Jesse M. 9 >> Rheumatic pains Father lO Lihan H. . 7 ,, II Alfred B. . 10 ,, 12 Edwin C. . 7 ,, 13 Sidney P. . 7 ,, 14 Eliza S. 10 ,, Rheumatic fever Father& mothe 15 Ellen M. . II ,, 16 Elizabeth R. II > > Rheu ma tic fever 17 James M. . 10 Rheumatic pains Mother (rh.fvr.) 18 Margaret P, 8 » I Mother's sister 19 Phoebe B. . II ,, Rheumatic pains 20 Caroline W. II ,, 21I James F. 8 > 1 22 Florence S. . 8 .. 23 Agnes D. . II - 24 Elizabeth C. 10 ,, 25 Louisa R. . 9 ,. Mother (rh.fvr.) 26 Marianne H. 9 " >« 27 Catherine S. 8 ,, 28 Louisa J. . 9 it Father 29 Mary B. . 5 Rheumatic pains 30 Robert L.i . 9 31 Emily P. . 8 32 Florence W. 8 Whooping-cough Mother 33 Alice T. 6 1 » 34 Anne T. 9 •• 1 Got sudden (very tempor ary)p aralysis of muscles f left shoulder, Jan uary 22, 1881. Abstract : 1st series . 52 [less i (No. 35) admitted twice] . .51 2nd series . 51 [less 2 (Nos. 5 and 13) admitted twice] 49 3rd series . 34 [less 2 (Nos. 21 and 28) admitted twice] 32 "137 132 ILLUSTRATIONS, 183 SUB-SERIES III. Heart Condition 2nd or 3rd Part of Body where Supposed Cause Result attack Commenced Mitral murmur 2nd Fright Well .. 2nd Left side Right hand Fright (both attacks) No cause 1 1 Regular Right side General Nothing entered " Mitral murmur Hands for these six , , Regular 4th General Left arm under this heading Improved Well Mitral General Fright ,, Irregular Arms and legs No cause — Regular General ,, W^ell " 2nd Arms General School examination Rheumatic fever •• Mitral 2nd , 1 No cause ,, Regular 3rd Left side Fright 1 1 y 1 General No cause II 3rd No cause — ist 1 1 " " from fright ,, J 1 No cause 1 1 Mitral ,, J J J, Regular 3rd Hands Fright from blow ,, Soft m. at apex General Fright ) 1 Not quite reg. 2nd >. ist fright, 2nd scold- ing at school 1 1 Regular 3rd II 2nd and 3rd attacks fright Fright )i Irregular 3rd II ist fright, 2nd and 3rd unknown Improved Regular 3rd .. ist fright, 2nd and 3rd unknown Well 5> 2nd Left side General (face No cause " Syst. murmur most affected) " II Regular Face & left side I! 1 J >i General , , ,, • 1 3rd Right side only General (hands ist fright, 2nd un- known, 3rd schoo " Irregular and right side most) Fright II Regular General School punishment ,, Remarks See 43 See 29 (2nd series); 1 84 - CHOREA ANALYSIS OF TABLE A, I. Age and Sex. Total number, 132. Boys, 33 ; girls, 99 ; \\z.. Of twelve years olcl : 2 girls. Ten and eleven years old : 10 boys, 34 girls. Nine years old : 7 boys, 20 girls. Eight years and under : 16 boys, 43 girls. 2. Previous Attacks of Chorea. and attacks, 27. 3rd ,, ic* 4th „ 3. 3. [a] Antecedent Whooping-cough in Choreic Children^. The point was ascertained in 120 cases. Of 28 boys, 4 had not had whooping-cough ; i.e. i in 6. ,, 92 girls, i6 had not had whooping-cough ; i in 5-8. [b) Antecedent whooping-cough in three independent series of cases of 132: each, taken consecutively without reference to particular illness : — In ist series, 27 of 105 had not had whooping-cough ; 27 no record. In 2nd series, 31 of no had not had whooping-cough ; 22 no record. In 3rd series, 33 of 104 had not had whooping-cough ; 28 no record. Hence it appears that 2 in 12 choreic children are with6ut history of whooping- cough, whilst 2 in 5 non-choreic children are without such history. Who&ping- cough has thus more than twice the frequency in the choreic over ike non~choreic. 4. Rheumatic Connection. In the ist Sub-series of 51 cases : — 1 had had rheumatic fever previously. 2 ,, ,, rheumatism previously. 4 were doubtful as to- this point. 3 were not ascertained. In the 2nd Sub-scries of 49 : — 4 had had rheumatic fever. 6 had probably had rheumatism. 2 were too doubtful to reckon. I was not ascertained. ANALYSIS OF TABLE A. 185 In the 3rd Sub-series of 32 : — 2 had had rheumatic fever. 7 ,, ,, pains, probably rheumatic, I was not ascertained. Hence 132 cases give 7 who have had rheumatic fever, 14 or 15 who have had pains, probably rheumatic ; 6 that were doubtful, 5 not ascertained. In 73 cases where that point is noted rheumatism is in \he family history of 19 ; of these 19, 9 have themselves had rheumatism, 10 have not. Of these 9 who have rheumatism, both themselves and in their famihes, rheumatism might have been the immediate cause of chorea in 2, and rheumatic fever in i (No. 14. Sub- series 3). [The above is as exact an account of the rheumatic connection as the facts admit of ; precise accuracy cannot be insisted on. See p. 33.] 5. Exciting Cause. Of the 132 cases : — Supposed exciting cause not entered in Not to be ascertained in ... . Rheumatism exciting cause in . . . Rheumatic fever exciting cause in Fright or sudden shock in . Some nervous strain, school discipline, hard lessons, &c., in Previous illness in 20 45 4{?3) 2(?I) 39 (? 2) 18 (?2) 4 [The figiu-es within the brackets denote proportion of doubtful cases under- each heading.] Hence it appears that of the 112 cases of which the exciting cause is presum- ably known, 57 or 53 ( say one half) have their origin in some notable nervous shock. 6. Implication of the Heart. Of the 132 cases : — No report as to heart condition in 13.^ In 2 cases of twelve years old there was heart defect in none. In 44 cases of ten and eleven years old there was heart defect in 16 (murmur in 13, irregularity in 3). In 27 cases of nine years old there was heart defect in 3 (murmur in 2, irregu- larity in i). In 59 cases of eight and under there was heart defect in 26 (murmur in 16, irregularity in 10). Hence, in 132 cases there is heart defect in 45, the largest proportioji of such defect appearing with the yoimgcst children. 1 In the majority of these, if not all, it may safely be assumed that there was no heart defect, and that express note of the heart was therefore not entered. CHOREA. Place of Origin of the Chorea as regards the Several Groups of Muscles. Of the 132 cases : — No report as to place of origin in ... . Chorea ' general ' from the first in ... . Chorea commenced in a particular group of muscles (whether or not becoming general after) in . 24 36 69 to 72 Locality of first onset distributed as follows : — Right side ...... Left side 13 times 15 .. ( ' Side ■ signifying generally arm and leg, but sometimes the arm only). Both hands 10 (or 11) time ,, arms twice ,, legs Once Right hand 10 time Left hand 4 •. Right arm 3 1. Left arm 6 , , ,, leg Once Right leg o Face 4 time Hence, taking sides of the body (whether arm, leg, or both chor was rigfit- sided in 26, left-sided in 26 ; taking upper limbs against lower, occupied the upper in 36 ( hand or hands in 25, arm or artns in 11), t lower in 2. TABLE B. 45 CONSECUTIVE CASES OF CHOREA ILLUSTRATING THE SAME POINTS AS TABLE A, BUT IN SOMEWHAT OLDER SUBJECTS. 1 88 CHOREA. ><5 in •a C/3 c o o 73 W 3 o (I) c o £3 O c o > .*_« o nS s S U, H, O o 13 S 3 N O J3 T3~ B 'S o U o '3 ■5 CQ -d c o o C/3 it a o O C/2 c o -a 3 a: o o >-> ■d to C », bO oJ O 'tr ♦- ii da Z> ^ ^' ^ .'SO c ^ o r- 1-, ^ w o s O '" 'S £ *"- Tj-'d T3 >" ^ "^ T3 -O b/D'in 0) ^ G ^ o P .- x: 6 ru HI S2 rt 5 ji -. 3 5 ^ 1 < i: 2 to C^ to m „ 00 m 1-4 M ri rn VO -t a^ C\ <; M HI HH M M y. >-r" ri X ClH* K ^ hl^ < rt lU -s J c rt 3 6 'S c < .Si 'S c CQ c c 3 3 B d C/2 < a d M CJ fO ■^ LO vO t^ 00 <7\ HI N fn ^ 55 1-4 •^ M M ILLUSTRATIONS. 189 iJ -§ c s o "K o c/2 ^ o > (/) — < c s — O 8 <^" i2 ^ ^3 .5 i .t: (u .0 -d |< _ Ci. pi rt^ .S ■n ts o Ti " T) Ci, (U 5 bJDrt £^ P-^j £ u c b/; 3 O s- s O U c o o 0) 7i C/2 b/) c i^ n ^ I-. =-) .4^ >t •^ Cl i^ bn is r: ■«^ br. ►v^ •— ' %i r; t-i fe ^ 0) -3 c in tn r^ S U .'-l rt ^ rt (U .-s a ^ (U n= o o tf S, Qj c . — in C h ;-, _r w o c S rt ^ o h (U G O o 5^ ^c2 T3 O §^ >^ "^ _ S S' !- ^* (-1 ^ V p 2 S >^^ °c ^ CD > w CI O £ >. OJ (L) bX) ■ o bfl .?^ b/] >> c ci >^^i- rC 0) a 'S s bo "^ ■5 ^ ■'^ ■"^ bfi ht side, ttack in ot fallin rt ^ bo ci & C/2 Gi C 03 " P ti O O c D I I ^ ci u f J >^ -t5' 'ri (N S P^ H M CM M 00 M tN M CI CO M H 00 M g-g • <■ CQ r/) c3- cS I- .y < J < lJ^ v,0 t^ CO ■^ M w H u SI O g Is •r -5 b 8 I90 CHOREA. £.2 I- C "1 c r: O E "5 c t^ '^ c o '-^ c 01/35 5 > c c o P 5 a <-> %» to •S O (/J s .50 t/! O ■^ 00 C 5hi 8 - 2 ^ o c/i o ;i S 3 > rt o « £ o >^ " t. .- c c > tJ3 c -a c o o 73 ^ ^ M ^ T3 >0 C •- o c o V^ 43 ui :73 ri o C aj •^ a % £ o " 2 il •^ o •SO Si .0 O OJ •- <;, re s "? .^ ^ 13 3 T3 XI ^ ./I o t, bo (u re Oi £ O* ro rt IS rt U « c £ u < w o C re c c < cii CU 8 ^ « B .n S S E W »/^ VO CO m w ILL USTRA TIONS. 191 < :S T3.2 - c > 00 o ^U ■55 o ^■^^ •Si}-" O O 0) 5;^ 0) c 2^ att mad ff joi ) TJ ■*-• -^n ^-d .-. ^^0 P j:: CD ^ -Z t; CJ i-, 3 •-.' > rt 3 ^ JD^ >^ ^ .- <-> OJ C >^ 0,3 -1^-^ aj Tj > b/) .2 b 3 .2 - >-i s ^^Q J5 ^ b/) c ^ aff ears eatn C .0 P>^ j b/: ^ O =^ 3 O 2; ,3 oi, i^ u CC ^ Si E^ OJ.P S bx 3 « 0) t*-! C ^ OJ rt ■i c, 3 r. cl 9^ ^ ^.s 'Zl 3 m U5 OJ C^ CO T5 T! rt ?5 3 ^5 (U ^ -^ H 192 CHOREA. ANALYSIS OF TABLE B. I. Age and Sex. Total number, 45. Boys, 10 ; Girls, 35. Over thirteen years : 3 boys, 7 girls. Twelve and thirteen years : 2 boys, 12 girls. Ten and eleven years : 2 boys, 9 girls. Nine years old : i boy, 3 girls. Eight years old and under : 2 boys, 4 girls. 2. Previous Attacks of Chorea. Second attacks : 11 Several previous attacks : i 3. Rheumatic Connection. Had had rheumatic fever, 4 ,, ,, rheumatism, 5 Were doubtful, 11 4. Exciting Cause. Rheumatism exciting cause in 2 Fright, or sudden nervous shock, in 2 Exciting cause, absent in 15 5. Implication of the Heart. No report as to heart in 21 ' Of the 10 cases over thirteen years old there was heart defect in 4 Of 14 cases twelve and thirteen years old ,, ,, ,, 4 Of II cases ten and eleven years old ,, ,, ,, 4 Of 4 cases nine years old , , , , , , i Of 6 cases eight years old and under ,, ,, ,, 3 Hence, in 45 cases there is heart defect in 16. ' In the majority of these, if not in all, it may safely be assumed tliat there was no heart defect, and that express note of the heart was therefore not entered. ANALYSIS OF TABLE B. 193 Place of Origin of the Chorea as Regards the Severat Groups of Muscles. Of the 45 cases : — No report as to place of origin in 2. Chorea general from the first in 5, ,, commenced in a particular group of muscles in 38. Locality of first onset distributed as follows : — Right side • 4 Left hand Left side • 4 Right arm . Both hands . 8 Left arm ,, arms . 2 ., leg ., legs . I Right leg . Right hand • 4 Face Two were examples of spasmodic twitching of head and shoulders. RESULTS FROM THE TWO TABLES TAKEN TOGETHER. X, Age and Sex. Total number, 177. Boys, 43 ; Girls, 134. Over thirteen years : 3 boys, 7 girls. Twelve and thirteen years : 2 boys, 14 girls. Ten and eleven years : 12 boys, 43 girls. Nine years old : 8 boys, 23 girls. Eight years old and under : 18 boys, 47 girls. 2. Previous Attacks of Chorea. Second attacks : 38 (more than one-fifth). Third attacks : 10. Fourth attacks : 4. 3. Rheumatic Connection. Had had rheumatic fever, 11. ,, ,, pains probably rheumatic, 20. Were doubtful, 17. Not ascertained, 5. 4. Exciting Cause. Rheumatism, acute or not, appeared to be exciting cause in 8 (4 of these doubtful). Previous illness, exciting cause in 4. Fright or some nervous shock or strain, exciting cause in 85 — nearly haif (4 of these doubtful). Exciting cause not to be ascertained, 60. ,, ,, not entered, 20. O 194 CHOREA. 5. Place of Origin as Regards the Several Groups of Muscles. Of tlie 177 cases : — Place of origin not entered or not known in 26. Chorea general in 41. Commenced in particular group of muscles in no. Locality of first onset distributed as follows : — Right side . . 17 Left hand • 5 Left side . . 19 Right arm . 4 Both hands . 18 (or 19) Left arm . 10 ,, arms . . 4 ,. leg 2 legs . . 2 Right 1^ I Right hand . . 14 Face . 9 Five cases indefin ite or anomalous. Hence, in 151 cases {i.e. excluding 26 not known) : Chorea was both sided in 65 (or 66). ,, ,, in the face in 9. ,, ,, one sided in 72. ,, ,, indefinite or anomalous in 5. Chorea affected the upper limbs in 55. ,, ,, the lower limbs in 5. ,, ,, the right hand 14 times, against the left hand c^ times ; but the right arm only 4 times, against the left 10 times. Of all parts of the body, chorea began most often in both hands ; while in either one or both hands it began in 38 instances, i.e. in more than a quarter of the total number whose place of commencement was known. INDEX. ACtr ACUTE chorea, 17 in connection with acute rheumatism, 42 treatment of, 137 case of, 158 Adult chorea, 21 cases of, 158, 168 Age of chorea, 19 Age-incidence of heart symptoms in chorea, 54 Age of fatal chorea, 75 Alcohol in treatment of chorea, 138 Anaemic murmurs simulating choreic, 53 Ancient chorea, 12 Aperients, use of, in chorea, 135 Aphasia, absence of, in chorea, 16, 98 Arsenic in treatment of chorea, 136 Associations of chorea, 28 Ataxy of chorea, 108 case illustrating, 157 BARNES on the pathology of chorea, 90 Bastian on the pathology of chorea, 88 Bouilly on the ' fever of growth,' 34 Bright on rheumatism as cause of chorea, 82 — on pericarditis as cause of chorea, 83 Bristowe on cardiac murmurs, 56 Broadbent on the pathology of chorea, 8g Bucheteau on imitative tendency of chorea, 29 DUR CAUSES, exciting, of chorea, 23, 76 tables of, 176 statistics of, 185-193 Change as treatment in chorea, 134 illustrations of benefit of in chorea, 140 Charcot on chorea of old age, 21 — on morbid anatomy of hemichorea, 93 Chauveau, experiments on dogs, 94 Chronic chorea, treatment of, 140 case of, 171 Convalescence in chorea, 141 DEBILITY, treatment of, in chorea, 133 Definition of chorea, 13 Deglutition, defects of, 16 Delirium in chorea, 28 Delusions in course of chorea, case illus- trating, 164 Dickinson on age in chorea, 19 sex in chorea, 20 recurrence of chorea, 26 rheumatic connection of chorea, 36 heart symptoms in chorea, 48 mitral reflux in chorea, 70 fatal chorea, 'j'j the morbid anatomy of chorea, 86 Diet in chorea, 132 Donkin, treatment of chorea by, 172 Drug treatment in chorea, 135, 163 Duration of chorea, 25 196 INDEX. EMB EMBOLIC theory of chorea, 78, 84 Emotion exchanged for movement in chorea, 117 — likeness of, to chorea, 14 — symptoms prominent in fatal chorea, 76. 79 cases illustrating, 156, 164, 171 Endocarditis with chorea, 46, 56 case of, 153 Epilepsy, connection with chorea, 29, ti6 n. Exciting causes of chorea, 23 tables illustrating, 176, 193 FACE, chorea of, 15 cases of, 155, 171 statistics of, 194 Family histor}' of rheumatism in chorea, 38 statistics of 185 Fatuity succeeding to chorea, case of, 171 Fatal chorea, illustrations of, 73 case of, 172 Feeding, difficulty of, in acute chorea, 138 cases illustrating, 157, 160 Ferrler on localisation of brain Injuries, 106 Freedom from restraint in chorea, 160 Fright, as cause of chorea, 23 — frequent occurrence of, 43 — statistics of, 185, 192, 193 GEOGRAPHICAL distribution of chorea, 22 Gowcrs on epilepsy in chorea, 29 mco-ordination in chorea, 113 Gray and Tuckwell on duration of chorea, 25 HAND affection in chorea, 114 cases of, 165, 169 Hayden on heart symptoms of chorea, 55 MOR Heart affection in chorea, 26, 44 cases of, 151 at various ages, 66 in connection with paresis, 69 case of, 164 — post mortem in chorea, 78 Hemichorea, 15 — case of, 167 Hemiplegia with chorea, 29 case of, 153 Hughes on sex in chorea, 20 Hypothesis as to heart signs in chorea, 70 Hysteria in connection with chorea, 28 — distinction from chorea, 161 — cases of in male, 164, 171 IMITATIVE tendency of chorea,'^29 case illustrating, 163 Immermann on dynamic murmur, 62 Incontinence of urine in chorea, 171 JACKSON, Hughlings, on pathology of chorea, 85 joint pains associated with chorea, 40 cases illustrating, 165, 166 Jones, Handfield, on the pathology of chorea, 91 K IRKES on embolism as cause of chorea, 78, 84 LIMBS selected by chorea, in — order of affection of, 114 — upper compared with lower in chorea, 186, 193 MACKENZIE, Dr. S., on pathology of chorea, 99 Mechanical support in treatment of chorea, 169 Morbid anatomy of chorea, 86 INDEX. 197 MOR Mortality of chorea, 72 Muscular strain as cause of chorea, 165 N ARCOTICS in acute chorea, 139 Nixon on dynamic murmurs, 61 OBJECTIONS to functional hypo- thesis of chorea, 124 Objects of treatment in chorea, 131 Ogle, John, on the blood in chorea, 90 Old age, chorea in, 21 Over-stillness in childhood, 28 Owen on rheumatic connection of chorea, 36 PAINS associated with chorea, 40, 135 Paresis, muscular, in chorea, 15 cases of, 165, 169 — cardiac in chorea, 68 Peacock on sex in chorea, 20 rheumatic connection of chorea, 36 Pericarditis as cause of chorea, 83 Poor, prevalence of chorea in children of, 144 Pregnancy, chorea in, 26, 90 case of, 167 Progress of chorea, 25, 134 Puberty, chorea of, 19, 75 cases of, 152, 164, 165 Pulse in chorea, 17 P3^e-Shiith on parts affected in chorea, 15 on age of chorea, 19 RECOVERY of chorea, 177 Recurrence of chorea, 26 significance of, 120 Rest in treatment of chorea, 131 Rheumatic connection of chorea, 32 tables illustrating, 176 statistics of, 193 Roger on connection of rheumatism with chorea, 35 • WHO Rokitansky on morbid anatomy of chorea, 92 Rosenstein on diagnosis of endocarditis, 56 SANSOM on the heart symptoms of chorea, 57 S6e on rheumatic connection of chorea, 35 parts affected by chorea, 15 sex in chorea, 20 recurrence of chorea, 26 Senator on heart disease in connection with chorea, 35 v. Side affected in chorea, 15 — changing from left to right, case of, 166 — statistics as to, 177, 193 Speech defects in chorea, 15 case of, 171 Steiner on rheumatic connection of chorea, 35 morbid anatomy of, 92 TEMPERAMENT in chorea, 19 Temperature lowered in chorea, 16 Todd, cases of hemiplegia in chorea, 30 — on pathology of chorea, 83 Tonic treatment of chorea, 134 Training of children, 143 Treatment, illustrations of, 157, 159, 169, 171 Trousseau on the rheumatic connection of chorea, 35 Tuckwell on fatal chorea, 74 VARIATIONS, rapid, in chorea, case illustrating, 156 Violent chorea, case of, 158 WEST on imitative tendency of cho- rea, 29 Whooping-cough, large proportion of, in chorea, 176 198 INDEX. 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