Chorea

CHOREA (from Greek choreia - dance), a hyperkinetic syndrome characterized by involuntary movements in the form of arrhythmic, uncoordinated muscle contractions, observed both at rest and during movements, not subject to voluntary inhibition, disrupting the correctness of active movements and disappearing during sleep. Corresponding to etiology, pathological anatomy, course, etc., several nosologically distinct forms of Chorea are differentiated. All these forms have as their basis an organic lesion of the brain, but the origin of the term chorea is historically connected with psychogenic disorders. In the Middle Ages, the term "chorea" was used to denote psychogenic, hysterical convulsions that often, under the influence of psychic epidemics, spread widely in the form of "dancing epidemics." Patients in groups, sometimes to music, performed movements similar to dancing. Since these "dances," which were nothing other than hysteria, could be cured under the influence of psychic influences, due to the prevailing religious sentiments of the time, these patients found healing after prayers and services in certain "holy places," at the tombs of saints, etc. In 1418, a large dancing epidemic was observed in Strasbourg, and according to the prevailing religious superstition, this dance could be cured at the chapel of St. Vitus in Zabern, to which these patients were sent by the Strasbourg magistrate. Hence arose the term "St. Vitus Dance," which is still used today as a synonym for the term "chorea." Although in that era other "dances" were also known (St. Anthony's dance, St. John's dance, etc.), the term "St. Vitus Dance" for some reason became particularly firmly established and was transferred into medical terminology along with the term "chorea." In medical literature, the use of this term begins with Paracelsus (1493-1541), who correctly defined the hysterical nature of epidemic dances; along with this form of Chorea, which he designates by the term "dance mania," Paracelsus drew attention to the existence of another, "true" (chorea naturalis) Chorea, under which undoubtedly non-hysterical forms of convulsions were meant. In 1686, the famous English physician Sydenham used the term "chorea" to denote the disease he described for the first time, known today as Chorea. A great merit of Sydenham was not only the establishment of a specific nosological form, but also that he isolated one form of convulsive disorders into a special group, which had previously been absorbed under the general term chorea st. Vite, which included the most diverse forms of convulsive disorders: tics, myoclonias, epilepsy, hysteria, simulation, etc. It should be considered unfortunate only that Sydenham used the old term "chorea" for the disease he described for the first time. Therefore, to distinguish this form of disease described by Sydenham, in addition to the term "chorea," terms such as "Sydenham's chorea," "little chorea," "English chorea," etc. began to be used (see below). This isolation of "Sydenham's chorea" into a special group proved all the more expedient that subsequently other forms of Chorea were described. Chorea convulsions represent only an expression of a focal brain lesion of a specific localization, and therefore the syndrome of these convulsions can be observed in diseases different in their etiology. Thus, along with Sydenham's chorea, which is an acute infectious disease, there are chronic forms of Chorea. This includes, for example, the so-called "hereditary chorea," first described by Huntington in 1872; it is a chronic progressive disease. Chorea convulsions can be a manifestation of infectious diseases of the brain; they are often observed in epidemic encephalitis. Cases of chorea convulsions in poisoning (carbon monoxide, iodoform) are known. Cases of chorea in pregnant women have been known since ancient times (see below). Chorea convulsions can affect only half of the body (hemichorea), being the result of a focal lesion (hemorrhage, syphilis, thrombosis) of certain areas of one cerebral hemisphere. Below are more detailed data regarding the main forms of chorea. Sydenham's Chorea [syn.: St. Vitus Dance, little Chorea (ch. minor), ordinary Chorea (ch. vulgaris), English Chorea (ch. anglorum)]. As noted by Sydenham, this disease is observed mainly in the second half of childhood; in girls twice as often as in boys; neuropathic constitution apparently represents favorable soil for the development of Sydenham's chorea; only in rare cases does the disease develop suddenly, usually it has a subacute gradual development. First of all, a certain speed, impulsiveness of all movements attracts attention; at the same time, individual twitchings appear in the limbs or facial muscles. Often at first parents take these contractions for "mischievousness" or "bad habit," but the increasing spread of convulsive twitchings and their intensification lead to the conviction of the pathological nature of these phenomena. In general, chorea convulsions are characterized by the following features: they are widespread, arrhythmic, uncoordinated, disorderly, involuntary, not subject to voluntary inhibition and disrupt the correctness of active movements; they are observed both in the proximal and distal parts of the limbs, intensify with excitement and voluntary movements, disappear during sleep; a characteristic accompanying symptom of them is hypotonia of the musculature. Corresponding to different groups of musculature, chorea convulsions manifest in the following forms. In the facial area they are expressed by various grimaces: wrinkling of the forehead, blinking, drawing apart of the corners of the mouth, protrusion of the tongue; sometimes convulsions spread to the musculature of the pharynx, larynx, soft palate, leading to disorders of swallowing and articulation. Contractions of the neck musculature manifest as nodding movements of the head, shaking, turns, etc. In the upper limbs various contractions are observed: flexion, extension, abduction, pronation, supination; twitchings of the shoulders are very common. All these movements can have different intensity: from barely noticeable twitchings when the patient is asked to hold hands stretched forward to sharp disorderly contractions disrupting the correctness of active movements, sometimes to the degree of complete inability to perform their function. Among the characteristic symptoms of Chorea from the upper limb, the so-called "chorea hand" should be mentioned: this symptom consists in the fact that when the upper limbs are stretched forward, flexion in the wrist joint and extension in the metacarpophalangeal and interphalangeal joints are observed. In the lower limbs the same involuntary contractions in the form of flexions and extensions in various joints, rotational movements of the feet, etc., are observed. Standing and walking are more or less difficult, sometimes to the degree of complete inability to perform these functions. Sometimes gait takes on the character of jumping (sautillement) - a condition resembling the so-called "saltatory convulsion," which is a hysterical disorder and consists of jumping appearing at each attempt of the patient to stand on his feet. The participation of the diaphragm and respiratory musculature in chorea convulsions results in an irregular, convulsive character of respiration. Involuntary contractions of the laryngeal musculature lead to the patient emitting moaning sounds. Speech difficulties sometimes reach such a degree that patients completely refrain from talking (choreic mutism). The intensity of convulsions depending on the stage and severity of the disease varies in different cases: gradual transitions from barely noticeable twitchings to sharp convulsions of the entire musculature in the form of the so-called "furious dance" (folie musculaire) can be observed. Muscle strength in Chorea usually does not present significant disorders. Only in certain forms, usually severe, paralytic phenomena are observed; these paralyses or pareses can manifest in the form of monoplegias, hemiplegias, paraplegias. Such forms of Chorea accompanied by paretic phenomena are designated by the terms "paralytic chorea" (chorea paralytica, limp chorea). Reflexes usually do not show deviations in intensity. As a specific change for Chorea, the knee reflex should be mentioned: the choreic Gordon reflex: tonic tension of the extensors of the knee joint when tapping the tendon of the quadriceps muscle (prolonged extension after eliciting the knee reflex). Among the usual phenomena for Chorea, disorders from the psyche should be mentioned: irritability, capriciousness, difficulty in concentrating attention. During sleep convulsions cease, but often they prevent the patient from falling asleep. A very serious complication is the development of psychosis in Chorea. These choreic psychoses can take various forms (confusion, manic states); depressive states are most often observed.

Very common complications of Chorea are endocarditis, rheumatism, angina—a circumstance of very great importance for explaining the pathogenesis of Chorea (see below). As rarer combinations of Chorea with other diseases, one can mention the combination of Chorea with tetany, hysteria, Basedow's disease, epilepsy. The combination of Chorea with epilepsy can manifest in various forms. Under epilepsia choreica (Bechterev) a special form of epilepsy was described, in which the convulsions during the attack have a choreic character. In other cases, the matter concerns the alternation of short periods of choreic convulsions with periods of epileptic attacks, and finally ordinary Chorea is encountered, during which separate epileptic attacks are observed. Among the skin changes in chorea, mention should be made of purpura, erythema nodosum, which represent the result of accompanying rheumatic and endocarditic changes. The same should be said about the temperature elevations sometimes observed in Chorea. Chorea by itself is not accompanied by fever; the temperature elevations are the result of endocarditis or rheumatism. The pathological anatomy of Chorea was established comparatively recently. In former times Chorea was classified in the group of functional diseases. The first indications of the organic nature of Chorea date back to 1908 (Mégus, Babonneix); their essence amounted to the dependence of Chorea on lesions of the large ganglia of the base, namely the striatal bodies. Further research and deeper knowledge of the function of the striatal system have fully confirmed this assumption, and at the present time the choreic syndrome should be regarded as one of the forms of striatal hyperkinesia. Sydenham's chorea represents an inflammatory lesion of the indicated area; the pathogenic factor of this inflammatory change is infection, which, in addition to encephalitis, leads to more or less pronounced manifestations from the heart and joints. The essence of the pathological-anatomical changes observed in Sydenham's chorea consists in vascular phenomena in the form of microscopic hemorrhages and softening and in foci of inflammatory infiltration [see separate table (Vol. XXXIII, pp. 551-552), Figs. 4 and 5]. These changes are localized in the basal ganglia and mainly in the lentiform nucleus. But along with this, as with any inflammatory process, other localizations are of course possible. This also explains the certain diversity of clinical manifestations of Sydenham's chorea. The diagnosis of Sydenham's chorea usually presents no difficulties: the specific character of the convulsions, their combination with changes from the heart or with rheumatism, the age of childhood, the presence in some cases of pyramidal symptoms—all this ensures the correct diagnosis of this disease. In hysteria, the convulsions do not possess all the features characteristic of Chorea; in choreiform convulsions developing under the influence of imitation in hysterical patients, there is no hypotonia, the symptom of the choreic hand described above is absent, there is no tonic form of the knee reflex (see above); in 'hysterical chorea' there are no organic symptoms of lesion of the pyramidal tract (Babinski's symptom, inequality of reflexes, flexion combinee), which are often observed in true Chorea due to the spread of the inflammatory process to the cortex, internal capsule, etc. The so-called paralytic form of Chorea usually develops in the presence of choreic convulsions. In rare cases, these convulsions are absent or are so slight that they may remain unnoticed. In such cases, difficulty in diagnosis may arise. The absence of signs of brain disease or lesion of the peripheral nerves, the frequent manifestations from the heart, the detection upon careful observation of slight isolated involuntary contractions of the musculature—all this makes it possible to distinguish 'choreic paralysis' from other forms of paralysis. In bilateral athetosis (ath6tose double) with hypertonia, the convulsions have a slow character and are more pronounced in the distal parts. Senile chorea and Huntington's chorea can be recognized by the age of the patients, the progressive chronic course. The prognosis in Sydenham's chorea can be considered favorable; usually the disease ends in recovery after 2-3 months, in rare cases it passes into a chronic form. A fatal outcome is observed in very rare cases and its cause is usually heart disease. The possibility of recurrences should be kept in mind. In the treatment of Sydenham's chorea, rest occupies an important place; in all cases complete bed rest is necessary; only in the mildest forms, during the recovery period, can one be limited to staying in bed for 3-4 hours during the daytime. In initial forms—even with a slight degree of convulsions, bed rest is mandatory. Among medicinal agents in the acute period—salicylates, urotropine, sedatives (valerian, bromine and luminal). Later arsenic preparations internally (Fowler's solution) or under the skin (Natr. cacodylicum). Among hydrotherapeutic procedures—tepid baths, wet wraps, of course taking into account the condition of the heart. In addition to Sydenham's chorea, the following acute forms of Chorea are known: chorea during pregnancy (see below), choreic convulsions in acute form in infectious diseases in case of their complication by encephalitis spreading to the striatal system. Most often, choreic phenomena are observed in epidemic encephalitis. The phenomena described under the name chorea electrica (Dubini's disease, Henoch's disease) represent two different diseases. Dubini's disease (chorea electrica Dubini), described by him in 1846, apparently also belongs to infectious diseases. It was observed in northern Italy and was characterized by short rapid contractions of the muscles, as if from electric shock. The disease affected people of various ages. The presence of high temperature, epileptic attacks, atrophic paralysis, fatal outcome constituted the main features of this disease, which has nothing in common with Chorea except for the name and, in view of the absence of anatomical research, remains unclear as to its etiology. As for Henoch's disease (chorea electrica Henoch), it is essentially one of the forms of myoclonia (see). The so-called chorea electrica Bergeron does not represent an independent disease form; the characteristic features of the disease described under this name are rapid twitchings of the limbs or head; this disease can supposedly be eliminated by emetics (0.05 Tart. emet.). Cases attributed to Bergeron's electric chorea represent hysteria or myoclonia, and this form cannot be ascribed nosological independence. The 'nocturnal chorea' (chorea nocturna) described by Oppenheim is nothing other than Sydenham's chorea. Under this term should be understood those very rare cases when choreic convulsions are observed only during sleep, in contrast to the usual disappearance of choreic convulsions during sleep; this 'nocturnal chorea' represents an extreme rarity. Along with the acute forms of Chorea described, there are forms of Chorea with a chronic course. This includes the above-mentioned Huntington's chorea (synonyms: 'chronic progressive chorea', 'hereditary chorea'). The disease is characterized by its hereditary-familial distribution. Remak described a family in which chronic progressive chorea was observed in 19 members. Huntington's chorea is inherited as a dominant trait: the absence of the disease in a given individual is a guarantee that it will not appear in his descendants. The disease develops between the ages of 30 and 40. The development is gradual. Clinically, Huntington's chorea is expressed by the involuntary movements described above in Sydenham's chorea; to this are added symptoms from the psyche in the form of progressive weakening of the intellect; at times attacks of excitement with hallucinations, tendency to suicide. The disease is incurable; death sometimes occurs many years after the onset of the disease with symptoms of cachexia and dementia. The pathological-anatomical basis of the disease is a progressive atrophic process localized mainly in the cortex, in the putamen and the caudate nucleus; the essence of this process is the breakdown of ganglion cells and the proliferation of glial elements; in the myelin fibers, changes described under the name status fibrosus (apparent increase in fibers due to their densification) are observed. The treatment of Huntington's chorea is limited to symptomatic measures. Senile chorea is nothing other than a manifestation of sclerosis of the cerebral vessels with predominant lesion of the area of the striatal body. The clinical picture of the disease consists of symptoms of chorea combined with other manifestations of sclerosis of the cerebral vessels. Under congenital chorea (chorea double) is meant a congenital choreic syndrome combined with general rigidity. Its basis is a congenital or acquired in infancy lesion of the striatal areas. Between congenital chorea and congenital athetosis there are gradual transitions and combinations ('choreoathetosis'). Finally chronic.

Chorea may occur on one side—hemichorea—as a result of unilateral lesions of the striatal area (vascular lesions, encephalitis, injuries). Hemichoreas may be combined with hemiplegias, i.e., with more or less clearly expressed symptoms of pyramidal tract lesions. Such 'symptomatic' hemichoreas are more frequently observed in childhood. Chronic forms of Chorea should include cases of transition of Sydenham's Chorea to a chronic form. Such forms represent a great rarity. It should be mentioned yet another form of chronic hyperkinesis, very close to the choreatic syndrome: this is the so-called polymorphic Chorea (syn. choree variable degeneres). Brissaud, who first described this form of the disease, characterizes it by the following features: 'these are cases of Chorea, which are not constant, intermittent, with a prolonged course. These are cases of Chorea, which do not differ either in the uniformity of their manifestations or in the regularity of their development or in the constancy of their course. Symptoms appear and disappear, increase and decrease, suddenly disappear and reappear unexpectedly'. Usually this form is observed in childhood, exclusively in children with a pronounced neuropathic constitution ('degenerates' chorea), with signs of infantilism. Symptoms of Chorea may remain for a lifetime, but usually they weaken in adulthood. The corresponding subjects suffer from symptoms of constitutional neuropathy (obsessive ideas, affective instability). All these facts indicate that 'polymorphic Chorea' represents a manifestation of insufficiency of the striatal system in the presence of a neuropathic constitution. According to the prevailing view at the present time, this form should be attributed not to the area of Chorea, but to the area of TICS.

M. Astvatsaturov. Chorea of pregnancy, a disease developing during pregnancy and, according to its clinical syndrome, hardly differing from chorea occurring outside of pregnancy. Chorea of pregnancy is a relatively rare disease, and moreover, as analysis of available data shows, its prevalence is not uniform geographically—in some localities it occurs more frequently, in others less frequently. Wilson and Preece (1932), in a collective statistics (951 cases, mostly from American obstetricians), state that chorea of pregnancy occurs approximately in 1:3,500 cases of pregnancy, in the Leipzig clinic it was encountered in 1:2,200 admissions (from 1886-1911, for 31,351 births), while in the clinic of Bodelot in Paris it was noted in 1:1,402 (from 1889-1899, for 15,638 births, 15 times). In the USSR it represents an extremely rare phenomenon; thus, in Pineles' (1910) collective world statistics out of 518 cases, only 3 were described in Russia (Weber, Bekhterev, Shchetkin). The rarity of chorea of pregnancy in the USSR is also confirmed by Selitsky. To date, only 22 cases have been described in the USSR; such a small number forces one to assume that by no means all observed cases were described. Mintz and Saltanov described another 5 cases of chorea of pregnancy—Chorea of pregnancy is more frequently observed in the north, while in the tropics, judging by available literary data, it does not occur at all. All cases in Russia (with the exception of one—Lazarevich) were observed in the north (the cases of Mintz and Saltanov were observed in the Kiev clinic of nervous diseases—all patients were Ukrainian women). There are no indications of possible influence of nationality, while Selitsky notes that all cases were observed in Russians and only once in a Jewish woman. The immediate cause of chorea of pregnancy is still unknown. Initially, chorea of pregnancy was considered completely isolated, no significance was attached to its first appearance only during pregnancy, it was treated as a random complication of pregnancy and classified the same as chorea infantum, hereditaria or senilis. Subsequently, a number of observations (reappearance of chorea infantum only with the onset of pregnancy, its first occurrence during pregnancy, recovery while still pregnant or after artificial termination of pregnancy and timely normal births) made it possible to tentatively speak of the influence of pregnancy as a predisposing factor to chorea; in recent times, in connection with the development of the doctrine of toxicoses of pregnancy, voices have begun to be heard that chorea of pregnancy represents one of the varieties of the toxemic processes of pregnancy. The emergence and strengthening of this view was facilitated by the inadequacy of existing theories and their lack of confirmation on clinical material (for example, the old theories of anemia, infection, etc.).-One of the first attempts at a scientific explanation of the etiology of chorea, namely its development on the basis of anemia, can hardly be considered justified at present time. If even the initial observations (predominant involvement of pale, anemic women, with a small number of red blood cells) remain valid and if indeed chorea is observed significantly more frequently in anemic women with an asthenic constitution, at the same time there are not sufficient grounds to see in this its etiological factor. Anemia definitely plays a certain role, but only an auxiliary role. And this anemia must be approached not as a specific anaemia gravidarum, arising ex tempore, but as anemia that is a consequence of constitutional insufficiency, which perhaps also causes lesser resistance of the blood during pregnancy and transition into more sharply expressed forms of the usual physiological and morphological changes of pregnancy. Another proof of the incorrect interpretation of chorea as a disease caused by anemia are by no means isolated cases of more severe and profound changes in the blood (anaemia, anaemia perniciosa gravidarum, diathesis haemorrhagica gravidarum, as well as various other changes in the blood observed before pregnancy and not directly related to it), which are almost never complicated by choreic convulsions. Of other theories, at one time the most widespread were the theory of embolism and the theory of rheumatism; rheumatism, as is known, is still by many authors ascribed predominant importance in the origin of chorea of pregnancy. The basis for the theory of embolism served the data found in the heart and brain at autopsy. Disorders of cardiac activity in the clinical course of chorea, especially in severe and advanced cases, are often observed and noted by many authors. Wilson and Preece, on the basis of analysis of 951 cases, state that sharply expressed cardiopathy occurs in */₃ of all cases and that in 87 out of 100 autopsies cardiac changes are found. Pointing also to changes in the brain (especially in corpus striatum), these authors tentatively express themselves about the identity of the lesions of the brain and heart. Despite this, however, there are not sufficient grounds to consider these lesions as the direct cause of chorea of pregnancy; it is much more correct to interpret these additional clinical symptoms from the heart, brain and psyche as a certain stage of the pathological choreic symptom-complex. What has been said above in relation to the theory of anemia applies equally to the theory of embolism and to the theory of combined changes of the heart and brain in chorea—clinical practice is rich in observations of various diseases of the heart, central and peripheral nervous system during pregnancy and does not note even relatively rare cases of combination of these lesions with chorea. Related to the embolic theory was the theory of rheumatism. It was widespread especially in France; its individual supporters considered chorea of pregnancy as a kind of 'rheumatic diathesis', as 'chronic cerebral rheumatism'. Roger (1867) drew a parallel between diseases of the heart and rheumatism, on the one hand, and rheumatism and chorea, on the other. Just as cardiac diseases are more or less connected with rheumatism, so persons who have had rheumatism sooner or later develop chorea. Roger, for this reason, believed that the pathological symptom-complex of chorea of pregnancy contains three components: lesions of the joints, heart and choreic convulsions. The theory of rheumatism was shared by many, is accepted by individual authors to this day, with only the difference that by some rheumatism is considered the primary cause of chorea of pregnancy, while others consider it only as a predisposing factor, and Preece, in analyzing his collected 951 cases of chorea of pregnancy, states that in more than */₃ ^{of all} cases in the anamnesis acute articular rheumatism was noted and that more than ^xli of all cases had rheumatism and chorea infantum. When calculating the percentage of mortality depending on various anamnestic data, these authors came to unexpected results. Thus, in women with chorea of pregnancy who had had chorea or rheumatism in the past, the mortality rate is 7, while in those who did not have these diseases in the anamnesis the mortality rate rises to 14-19 (this fact seems very significant, it cannot be explained even by Wilson and Preece; they only cautiously and tentatively express the opinion that the lower mortality may be allegedly due to some immunity formed after rheumatism or chorea infantum). From its very inception, the theory of rheumatism had opponents and encountered quite weighty objections from major authorities (for example, Charcot). One of the objections was that by no means in all cases of chorea of pregnancy was rheumatism observed in the anamnesis. This objection remains in full force to this day and indeed in not a few subsequent reports and descriptions of recent times (for example, in Selitsky) in the anamnesis of pregnant women with chorea, no past rheumatism is noted. Of course, in our time it is hardly possible to explain the very essence of such a multifaceted disease as chorea of pregnancy by rheumatism, all the more so since the immediate cause of rheumatism itself remains far from clarified. It is impossible to deny the possibility of influence (indirect) of rheumatism, but it must be approached in the same way as many other predisposing factors. Rheumatism, as well as other infections of early and late childhood and later periods (even adult age), has considerable importance in the delay of development of the organism and its subsequent inadequacy, especially at the moment of greatest demands on it. These infections can also cause a number of complications and deviations during pregnancy, but from this, however, one cannot conclude that every pregnant woman who had an infection in the past will develop chorea or some other toxemic disease. Even less justified are the infectious theory, the theory of reflex neurosis (irritation of plexus utero-ovarialis-Spiegelberg, Fehling and others) and the explanation of the occurrence of chorea of pregnancy by intestinal intoxication.

Attempts to find a specific causative agent have not been successful, and it is hardly likely that the immediate clarification of the nature of chorea of pregnancy, just like eclampsia, will be connected with the discovery of any specific 'chorea-virus,' while the finding in individual cases of various microorganisms can hardly indicate an infectious origin of chorea of pregnancy, but can only speak of a superimposed infection. The explanation of chorea of pregnancy by mechanical influences on the brain, brain tumors, increased function of the cerebellum, capillary embolism of the thalamus opticus and corpus striatum and others has also not gained widespread acceptance. At present, the theory of auto-intoxication (see Pregnancy, Pathological) appears more substantiated, which arose at the end of the 19th century and is now shared by many authors. This theory has its starting point in pregnancy itself, the fertilized egg, and the new biochemical relationships in the organism associated with its implantation. Initially, the view arose of poisoning of the organism by toxins coming from the fertilized egg, then attention began to be paid to the placenta, there were attempts to explain chorea, as well as other toxicoses of pregnancy, by phenomena of hepatotoxemia, metabolic disorders, dysfunction of individual or a number of endocrine glands (e.g. the theory of Simonini, which found many followers in Italy, that chorea is caused by insufficiency of the parathyroid glands). The basis for the toxemic interpretation of chorea of pregnancy were investigations of other toxicoses of pregnancy, the almost regular appearance of chorea at certain times, during the period of placental development (Birnbaum), successes in therapy, especially various kinds of sera, and the course of the disease after childbirth or artificial interruption of pregnancy. All this shows that if at the present moment one cannot categorically assert that chorea of pregnancy represents one of the many varieties of toxicoses of pregnancy, then in any case all the accumulated observations allow one to consider the toxemic theory the most probable. Clinic also to a large extent supports these views. The toxemic nature of chorea of pregnancy can be indicated by: the predominance of the disease in primigravidas (Kroner-69%, Gettkant 78%), its appearance predominantly at a younger age, at certain times of pregnancy, rare cases of chorea puerperarum and lactantium and others. In addition, one can note (apparently in connection with a certain asthenic constitution) the presence of toxic idiosyncrasy and the existence of a subspecies of so-called cumulative toxemia (see Pregnancy, Pathological). Chorea of pregnancy, in contrast to some other toxicoses (e.g. eclampsia), has a special tendency to recur in subsequent pregnancies (toxic idiosyncrasy). Moreover, in chorea of pregnancy, as well as in toxemic processes of pregnancy, a combination with other toxic pathological symptoms (with eclampsia, albuminuria, parenchymatous hepatitis, nephritis, vomiting, intractable vomiting, toxicodermias and dermatoses, cerebro- and psychopathia toxica and others) can often be observed. The predominant type of chorea of pregnancy is chorea bilateralis (this was already pointed out by Romberg), monoehorea is a very rare phenomenon. Chorea seizures arise either suddenly, abruptly, or their appearance is preceded by a number of prodromal symptoms (increased sensitivity, irritability, fluctuations in the psyche, changes in gait, in muscular sense and others). The appearance of the latter however is not necessary and far from rare chorea sets in suddenly without any precursors. The disease usually begins in the first weeks of pregnancy (according to Jaccoud, Barnes, Charpentier, Delage and others more often in the third month), much less frequently in the second half, while cases of purely postpartum chorea are extremely rare (according to Pineles, in 3.8%). Twitchings begin in the upper limb (initially isolated), less often in the lower, in rarer cases twitchings of the musculature of the whole body are observed from the very beginning of the disease. The character, intensity, as well as frequency of individual choreic movements are extremely diverse. Along with mild, relatively insignificant twitchings, often such strong ones are observed that it is difficult to hold the patient. The strength of twitchings and their frequency are by no means always an indicator of greater or lesser severity of the disease. Fairly significant intervals between individual attacks can also be observed and finally the course of chorea can be of a chronic nature. The intensity of twitchings can fluctuate under the influence of a whole range of different agents-movement of the fetus, labor pains, application to the breast and others. At night time even in severe cases twitchings either significantly decrease or even cease completely. In individual cases various other symptoms can also be observed, and they are expressed all together or some symptoms may be absent. Thus, Selitsky noted in chorea of pregnancy the symptom of Thomas (the affected arm, lowered down and raised to the vertical, is in sharp pronation), the symptom of Grasset (when sitting at a certain height when raising and holding the right and left leg the affected limb soon drops and falls), the symptom of Babinski (on the horizontally placed palms of the examiner the patient puts her own palms placed vertically; when producing rhythmic pushes of the palms of the patient the affected hand quickly passes into a state of pronation), the symptom of Gower (the legs of the patient are placed on the palms of the examiner; when alternately raising the right and left leg the heel of the affected leg produces less strong pressure), the symptom of Strümpell (the patient in the lying position with extended legs alternately bends the legs in the knee joint; the examiner counteracts this by pressure with the palm on the front surface of the thigh-at this time the medial edge of the affected foot demonstrably rises) and a number of other phenomena (falling backward with eyes closed, inability to stand with eyes open neither on the right nor on the left leg, lowering of the affected arm with arms extended forward both with eyes open and closed). The prognosis of chorea of pregnancy-always remains serious, because in chorea (especially in the absence of any other toxic symptoms) it is much more difficult than in other toxicoses of pregnancy to assess the severity of the process and transitional moments. In any case the prognosis is much more favorable than was thought earlier, and the percentage of mortality is now significantly lower (thus, if in old statistics the percentage fluctuated from 20 to 40, now it fluctuates from 3 to 7-10, while with some authors it is even 0). The immediate causes of greater or lesser severity of the disease are not yet clear; the view of some authors (e.g. Schrock's) that most favorably cases of chorea arising for the first time only at the end of pregnancy are contradicted by others; the point of view on the course of recurrent chorea of pregnancy is also contradictory-Kruger (Kriiger) for example believes that there is no reason to set a worse prognosis for it. Dieckmann, Hannes and others speak in the opposite sense. Pregnancy in the arising chorea does not always go to term [according to Charpentier in 161 cases in 72%; according to Delage (1898) in 143 cases in 70%; in Bamberg's in 64 cases pregnancy went to term in 33 cases, spontaneous abortion occurred 10 times, premature labor 5 times and in 2 cases pregnancy was interrupted artificially; in Selitsky's 7 personal observations only in one case was an artificial abortion performed and that in the first observation, in 1913]. Chorea of pregnancy in general very much predisposes to interruption of pregnancy and often leads to miscarriage or spontaneous labor, for which reason the prognosis for the fetus always remains serious and unfavorable, malformations and underdevelopment of the fetus (hydrocephalus, spina bifida) have also been described. Indication for interruption of pregnancy are severe cases, cases with a number of additional sharply expressed toxic symptoms, while in mild cases there is no reason to resort to abortion (Selitsky, Wilson, Price). The arsenal of proposed and proposed for the treatment of chorea of pregnancy means is very diverse. Along with a number of tonica, antispasmodica, antipyretica, laxantia, cardiaca, nervina, hypnotica, narcotica, hydroelectrotherapy, bloodletting and others were proposed. In recent time there is a marked tendency to limit the use of narcotics and to strive for the introduction, as with other toxicoses of pregnancy, of various means that seek to act not on individual symptoms but on the whole organism (solutions of grape sugar, serum of pregnant women, N-serum of horses). Along with one or another therapy, the way of life and diet of the patient are of no less importance.-Prevention of chorea of pregnancy is the struggle with early marriages, early pregnancy, in those who have had chorea during pregnancy-longer intervals of motherhood (to avoid cumulative intoxication).

Seditsky.