CHOREA (chorea; Greek choreia dancing) — the form of a hyperkinesia which is shown the bystry, random, spasmodic, not stereotypic, violent motions of various muscular groups.
The term «chorea» offered Para-tsels. Earlier as a synonym applied the term «Saint Witt's dancing» connected with the legend about treatment of the patients who had hysterical spasms at Saint Witt's chapel in Tsaberna (France).
In the course of studying hron. a giiyer-kinesis (see Hyperkinesias) the following nosological forms were allocated: the hysterical chorea described
by T. Sydenham in 1686 (see Rheumatism) I, and a chorea of Gentington — the hereditary progressing disease, in a wedge, a picture to-rogo a hyperkinesia is combined with deep mental disorders (see Gentington a chorea). Other choreic hyperkinesias in a crust, time treat as the syndromes developing at encephalitis (see), craniocereberal injuries (see), cerebral palsies (see. Children's paralyzes), at pregnancy (see), etc. Development of choreic hyperkinesias
is the cornerstone patol. changes of a striate body (see. Basal kernels). As a rule, patol. process, as a result to-rogo neurons of a striate body perish, extends also to other departments of a brain which are functionally connected with a striate body. Character and localization patol. process are defined by features of this or that disease, degree of manifestation and prevalence of diffusion and focal changes of a brain. At a chorea of Gentington and a small chorea considerable changes of neurons of a striate body are observed that leads to death of the last, and sometimes and nerve fibrils. At the same time other structures of a striate body remain that is regarded as an incomplete necrosis of this department of a brain (see the Brain, pathological anatomy). Preferential small neurons of a striate body at relative or full safety of large neurons and the expressed proliferation of neyrogliotsit perish. At the chorea caused by disturbances of cerebral circulation (see) focal changes (heart attacks, hemorrhages, incomplete necroses) and changes which are quite often extending and on other departments of a brain are characteristic (at the same time the hyperkinesia is combined with other nevrol. symptoms). Cases of development of a hemochorea are known (see below) at the heart attacks of a brain which are localized in cerebral hemispheres and destroying a body having a tail. The choreic hyperkinesia is sometimes observed at destruction of red kernels and upper legs of a cerebellum, thalamus (is more often with involvement in patol. process and striate body). The choreic hyperkinesia is observed at localization of a zone of a heart attack in the pool of branches of a back brain artery (see. Cerebral circulation). Also cases of a choreic hyperkinesia at multiple superficial hemorrhages in bark of a cerebellum are described (see) and at a bilateral subdural hematoma (see. Subshell hemorrhages).
At a chorea the muscles located on considerable removal from each other and various on functional purpose at the same time can be reduced. Various irritations, especially emotional, strengthen a choreic hyperkinesia, at rest it decreases, in a dream — disappears. The choreic hyperkinesia complicates autokinesias. At sharply expressed choreic violent movements there are impossible usual motive stereotypes — walking, a stoyanrga, purposive movements of hands, etc. The tone of muscles at a chorea is usually lowered. Tendon and cutaneous reflexes are, as a rule, kept, patol. reflexes are not characteristic. The exception is made by a hemochorea, an inborn and senile chorea (see below), at to-rykh often are found patol. reflexes (see Reflexes pathological), and the tone of muscles happens raised.
The choreic hyperkinesia, as a rule, is not the only thing a wedge, a symptom of a disease. Usually at these or those damages of a brain it is combined with other symptoms.
The present article is devoted to rather rare and isolated forms of a chorea — to a chorea of pregnant women, an electric chorea of Dubini, an epileptic chorea, a convulsive chorea of Morvan, a paroxysmal ho-reatetoz of Maunt — Riback, a high-quality hereditary chorea, a hemochorea, an inborn, senile and polymorphic chorea. Names of these forms reflect an originality a wedge, manifestations of a choreic hyperkinesia or are traditionally developed. Because the etiology and a pathogeny of a basic disease, against the background of to-rogo arises a chorea, are not always found out, treatment of many of these hyperkinesias has generally symptomatic character. The current and the forecast of the majority of the forms of a chorea given below depend hl. obr. from a basic disease.
The chorea of pregnant women arises more often at the first pregnancy at young people, especially adynamic, women, only in rare instances develops at the subsequent pregnancies. Organic lesion of c is its cornerstone. N of page with preferential involvement of extrapyramidal system (see). In 1867. Roget (N. of Roger) noted communication of a chorea with rheumatism and a frequent combination of a choreic hyperkinesia to damage of heart and joints. Zh. Zharko considered that the chorea of pregnant women represents one of forms of toxicosis of pregnant women (see), and rheumatism considered as the contributing factor. In the anamnesis most of patients has instructions on rheumatism; besides, signs of rheumatic damage of heart and other somatic displays of rheumatism can be noted (see). However communication with rheumatism manages to be tracked not in all cases of a chorea of pregnant women. Approximately in V3 of cases of a trochee of pregnant women is a recurrence of the hysterical chorea postponed in the childhood.
A certain polymorphism of structural changes in a brain is characteristic of a chorea of pregnant women, to-ry it is caused by degree of manifestation, localization and prescription of destructive changes in it, and also their frequent combination to pathology of internals. The Nek-ry researchers considering a chorea of pregnant women as an exacerbation of rheumatism or a recurrence of the hysterical chorea postponed in the childhood emphasize that it is often combined with an endocarditis (see), myocarditis (see), a pericardis (see), acute joint rheumatism. The most continuous variations are found in a striate body, and also in a thalamus, podbugorny area, black substance, bark and white matter of big hemispheres. There are considerable disturbances of structure and death of neurons, preferential small, to formation of the centers of an incomplete necrosis, a gliosis (see), perivascular lymphocytic infiltrates, and also to development of white (ischemic) and red (hemorrhagic) heart attacks (see the Stroke) as a result of thrombosis and Ekhmboliya of vessels of a brain. A number of researchers note defeat of a striate body (kernel preferential having a tail) at a chorea of pregnant women and emphasize absence morfol. symptoms of rheumatism.
The wedge, signs of a chorea of pregnant women appear in the first or in the second trimester of pregnancy. The violent movements at this form of a hyperkinesia same, as at a small chorea. However the overall picture of a disease is, as a rule, much heavier: hyperkinesias are expressed more sharply, changes of mentality meet more often and are more expressed. The chorea of pregnant women usually develops gradually. Symptoms harbingers are the uneasy dream, an acrimony, the general weakness, headaches, emotional lability, sensitivity, tearfulness. In several days violent reductions of muscles appear, and in the beginning one of upper extremities and a face usually is surprised. Then hyperkinesias take other extremities and a trunk, their amplitude increases. Patients are in a condition of alarm, concern, speak much, despite disturbances of an articulation. The muscle tone is lowered. The violent movements amplify at emotional loadings and purposive movements, during the walking. At rest the hyperkinesia decreases, in a dream disappears. At a number of patients symptoms harbingers are absent, and the hyperkinesia develops sharply. Having appeared in one of extremities, the violent movements in several hours take other extremities, and also a face, language, a trunk; their amplitude quickly increases.
At a chorea of pregnant women often there are mental disturbances. Activity of patients becomes chaotic, fussiness, euphoria, bystry change of mood are characteristic. Quite often excited state is replaced by a depression. Sometimes there are attacks of «a motive storm» (patients try to run somewhere, escape, incoherently speak). After the termination of an attack of psychomotor excitement intensity of hyperkinesias quite often on a nek-swarm time considerably decreases.
At inspection of patients it is possible to reveal a number of characteristic symptoms. So, in a standing position blindly (see Romberg a symptom) the patient falls back, and at open eyes cannot stand on one leg. In a sitting position, in attempt of the patient to raise both legs, on the party of defeat the leg sharply falls (a symptom Grass). Also Thomas's symptom — sharp pronation of a brush of the hand raised up is observed.
The current of a chorea of pregnant women and its outcome are defined by degree of manifestation of a hyperkinesia, hypotonia of muscles, the phenomena of the general exhaustion of mentality and symptoms of intoxication. In mild cases and at moderately severe pregnancy is possible to keep currents of a chorea more often, and childbirth comes to an end safely. At a heavy current of a chorea of pregnant women quite often there is an abortion (see Abortion) or there come premature birth (see). The fruit at the same time is, as a rule, impractical.
Treatment of a chorea of pregnant women complex. Appoint a high bed rest and absolute rest as any irritating factors strengthen a hyperkinesia. Food of patients shall be various, vitamin-rich, contain limited amount of carbohydrates. Drug treatment includes antirheumatic drugs and sedatives. Along with the purpose of a ginosensibiliza-tion use antihistaminic drugs. Use vitamins of group B, and also ascorbic to - that and Rutinum. For removal of a hyperkinesia and psychomotor excitement appoint neuroleptics, phenobarbital and bromides. Drugs are not recommended to pregnant women. In the presence of symptoms of intoxication (see) use the funds allocated for fight against toxicosis of pregnant women (see). At the expressed mental disturbances resolve an issue of the direction of the patient in a psychiatric hospital.
Abortion is shown in cases of a heavy and persistent current of a chorea at inefficiency of the carried-out conservative treatment, existence of intensive hyperkinesias, the expressed mental disturbances and signs of a decompensation of cordial activity. After abortion hyperkinesias not always completely are eliminated though their intensity considerably decreases. In this regard after abortion, and also normal childbirth it is recommended to continue complex conservative therapy.
Forecast, as a rule, favorable. In rare instances the chorea does not disappear after the delivery.
It is considered to be that the women who transferred a hysterical chorea in the childhood of a do'lzhna to avoid pregnancy at young age. Repeated pregnancy is contraindicated to the women who transferred a chorea at the first pregnancy within several years.
Electric chorea of Dubini. This form of a chorea is described by Dubini (A. D Tibini) in 1846; the name «electric» is connected with the fact that manifestations of this hyperkinesia remind reductions of muscles at irritation electric current. The acute onset of the illness, high temperature, existence of epileptic seizures, atrophic paralyzes is characteristic. The so-called rhythmic hyperkinesia coming at the same time in many muscular groups and sometimes passing into the general epileptic seizure is characteristic of an electric chorea of Dubini. Current heavy. Symptomatic treatment. Forecast adverse.
The epileptic chorea is described in 1897 by M. Bekhterev. It is a special form of epilepsy. Spasms during an attack have character of a choreic hyperkinesia. Unlike a hysterical chorea, the violent movements at an epileptic chorea arise only pristupoobrazno, are followed by the loss of consciousness and other symptoms characteristic of epilepsy (see). There are no changes of heart, typical for rheumatism. However the combination of an epileptic chorea to rheumatic damage of a brain is possible that is shown by alternation of the periods of choreic hyperkinesias with the periods of epileptic seizures.
Symptomatic treatment; use anticonvulsant drugs, tranquilizers. At rheumatic defeat of c. N of page carry out also antirheumatic treatment. The forecast for life favorable.
Convulsive chorea of Morvan. Develops at encephalitis. It arises at severe forms of encephalitis against the background of a hyperthermia (see. Gui-pertermichesky syndrome), vegeto-vascular dystonia, disturbance of a trophicity and metabolism. Myoclonias can join choreic hyperkinesias (see), generalized toniko-clonic spasms are quite often observed (see) with development of coma (see Côme)
Treatment is directed to a basic disease. The lethal outcome at not stopped convulsive status is possible and gipertermiches - which to a coma.
The paroxysmal choreoathetosis of Ma-unta — Riback is described in 1940. It is inherited on autosomal type of dominants nomas, appears at children's age. The disease is characterized by periodic attacks of a choreoathetosis of the person and distal departments of extremities. Attacks last of several minutes till several o'clock, can repeat several times in days. At adults they are provoked by smoking, alcohol intake, coffee, overfatigue. In the mezhpristunny period any nevrol. deviations do not come to light. A symptomatic treatment, the therapeutic effect as a result of reception of phenobarbital is noted. The forecast for life favorable.
The high-quality hereditary chorea is described in 1967 by Hererom (A. F. (Nayegeg) with sotr. The disease is inherited but autosomal dominantly to type, the pathogeny is not clear. It is characterized by a generalized choreic hyperkinesia with iytentsion-ny trembling. The disease does not progress and is not followed by mental disturbances. Symptomatic treatment. The forecast for life favorable.
The hemochorea (a chorea of one half of a body) is for the first time described by V. M. Bekhterev. Arises at unilateral damage of a striate body, hl. obr. vascular genesis. The hyperkinesia covers the half of a body opposite to the center of defeat. Treatment by the vasodilators and drugs improving metabolism in a brain can lead to improvement of a condition of the patient or to disappearance of a hemochorea.
An inborn chorea — the syndrome which is shown a bilateral choreic hyperkinesia to similarly bilateral athetosis (see). The inborn chorea arises right after the birth of the child and is caused by defeat of subcrustal nodes in the pre-natal period or at the time of delivery. Unlike an athetosis (double athetosis) it is characterized by a hyperkinesia of choreic, but not athetotic type, extra pyramidal increase in a tone of muscles — rigidity. A symptomatic treatment, tranquilizers, muscle relaxants, LFK, massage appoint. The forecast for life favorable.
The senile chorea develops at elderly people because of cerebral atherosclerosis with disturbances of cerebral circulation. In tissue of a brain various combinations patol are found. changes, napr, heart attacks in white matter of a frontal lobe, the centers of death of small neurons of a striate body, diffusion depression of white matter of parencephalons, proliferation of astrocytes; changes of small neurons of a striate body with a heart attack in a shell of a lenticular kernel, proliferation of neyrogliotsit in a striate body and bark of cerebral hemispheres; an atrophy of a brain with death of neurons and proliferation of neyrogliotsit in a striate body; death of neurons in bark of a back central crinkle with proliferation of neyrogliotsit, a heart attack in a thalamus and lack of changes in a striate body. The senile chorea has a nek-swarm looking alike a chorea of Gentington, but differs from it in what begins at more advanced age, Has no hereditary character, has more monotonous current and can not be followed by frustration of mentality, typical for a chorea of Gentington.
Treatment is directed to correction of cerebral microcirculation and metabolism of a brain, appoint vasodilating drugs, biostimulyator. At a senile chorea tendency to delay and even suspension of process is often noted.
The polymorphic chorea is for the first time described by E. Brissaud. According to M. I. Astvatsaturov, it is connected with inborn inferiority of striatal system. Hyperkinesias arise unexpectedly and also unexpectedly disappear, at the same time neither pattern of a current, nor monotony of manifestations is noted. The disease is observed at children's age, usually against the background of infantility (see), characterized by a long intermittent current.
Symptomatic treatment. At mature age hyperkinesias, as a rule, weaken, but can remain expressed during all life.
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