Chorea is a blasphemy. Horea Huntington and other species

Chorea is one of the forms of hyperkinesis and is a pathology of the nervous system, which is accompanied by malfunctions in the subcortical nodes of the brain and is expressed by chaotic, aimless and episodic twitching mainly in the legs and hands (less often in the trunk).

This term has a dual application. They also identify independent diseases from this group (such as Huntington's chorea or small chorea), and some syndromes that occur in a variety of pathologies. The name "chorea" is also not accidental: in translation from the Greek language it means a certain kind of dance. The patient during the attack looks really awkward: the fast, steep, uncoordinated and jerky movements of limbs and trunks create the impression of a disorderly, somewhat sweeping and involuntary dance. Therefore, chorea acquired another "historical" name - "The Dance of St. Witt", who came to us from the deep Middle Ages. By the way, many people have heard about this uncommon term, which in no way are involved in neurology. The fact is that St. Witt, who lives in Sicily, was tormented by the Romans in his time as an active propagandist of Christianity. And later the belief spread that every person who will dance before the statue of this Saint on his birthday (June 15) will receive a huge amount of energy and vigor for a minimum of one year. So, on this day, around the statue, superstitious citizens began to crowd around, who for their health did not hesitate to "give out" to ridiculously expressive dances. Hence the roots of the expression "the dance of St. Witt", which in due course was christened chorea, grows.

The causes of chorea may be different, but often they are hereditary factors. There are four varieties of this pathology, which became "hostages" of bad genetics (the so-called primary forms). It's about Huntington's chorea, Lesha-Naikhan's disease, neuroacanthosis and benign hereditary chorea.

Among other provoking factors of chorea, doctors indicate the following reasons:

  • Diseases of infectious nature (pertussis, borreliosis, neurosyphilis, HIV, viral encephalitis, tuberculous meningitis).

  • Metabolic disorders. In this group, the number of pathologies "culprits" is even richer: Sturgeon-Weber disease, hyperthyroidism, glutaric aciduria, Gallervorden-Spatz disease, hypocalcemia, metochromatic leukodystrophy, Wilson-Konovalov's disease, mucopolysaccharidosis, gangliosidosis, Lee's disease, homocystinuria, Merzbacher-Pelitseus disease , hyperglycemia, Fabry disease, etc.

  • Intoxication. Fatal may be poisoning with levodopa, various neuroleptics, digoxin, lithium, mercury and even oral contraceptives.

  • Autoimmune pathologies. The cause of chorea can be multiple sclerosis, systemic lupus erythematosus, a reaction to immunization, an antiphospholipid syndrome, etc.

  • Structural damage to the brain. Among them - strokes, various craniocerebral injuries, hypoxic encephalopathy and tumor formations.

  • All varieties of chorea, provoked by any factor in this group, are secondary forms of the disease. In addition, there is another type of chorea - psychogenic (it is a kind of psychogenic motor disorders).

    Common signs of chorea

    Chorea manifests itself as brief, chaotic and, as it were, accidental bodily movements, especially in the limbs. Sometimes they resemble the fast and perfectly normal movements of a healthy person who is disturbed by something, but they are not part of any adequate planned actions. In other cases, arbitrary shudders with chorea are similar to an uncontrolled and emotional "dance." These jerky jerks can be one- or two-sided, and even with the "participation" of both sides of the trunk they are not synchronized.

    Khorea Dance of St. Vitus

    Manifestations of chorea vary depending on the form of pathology. Thus, with slightly expressed choreic hyperkinesia, there is a slight motor anxiety, which can be accompanied by inadequate gestures, unexpected fussy movements, certain grimaces, increased emotionality and motor disinhibition. As for the clearly expressed forms of chorea, they usually manifest themselves in the peculiar movements of the "devil on the thread" or loosened up violent "dance". Expressive choreic hyperkinesis distorts not only speech and facial expressions, but even movements when walking. The walk of such a sick person can be described as strange and somewhere "clownish". And patients with an extremely severe form of chorea, in contrast, can not at all perform any movements. They find it difficult to move around because of permanent falls, they are not able to fully serve themselves in everyday life, and therefore they are dependent on close people. Choreic twitching in mimic and oral muscles of the face (including the larynx and tongue) prevent both verbal communication and food intake. Such patients can easily carry a fork with food past the mouth or can not "get" a napkin into the nostrils to blow out a stuffy nose.

    Types of chorea and their features

    1. Primary forms:

  • Horea Huntington. This disease can occur at almost any age, but usually develops in 35-45 years. Characterized not only by typical hyperkinesis, but also by dementia (decreased intelligence) and personality disorders. The development of the disease is gradual, sometimes it is even difficult to catch the appearance of the first violent movements. As a rule, they arise in the face area and look like random motor automatisms (opening the mouth, licking the lips, frowning, protruding the tongue, etc.). In the course of progressing pathology, hyperkinesis develops in the upper limbs (the so-called "fingers playing the piano"), and also extends to the trunk, violating the statics and gait. Even further, speech and swallowing processes, memory and self-service level deteriorate. As a consequence, there is dementia.

  • Sometimes Huntington's chorea starts with mental disorders in the form of behavioral, affective or hallucinatory-paranoid disorders.

  • Neuroacanthocytosis. In addition to the choreic hyperkinesia, it is also manifested by acanthocytosis (ie changes in the form of erythrocytes). The main difference of this disease from the previous one is that with it there is weakness and atrophy in the muscles of the hands and feet. It is also characterized by oral hyperkinesis with chewing movements, popping out of the tongue, twitching of the lips and other grimaces. Another distinctive feature of neuroacanthocytosis is involuntary biting of the lips, tongue and inner surfaces of the cheeks (up to the blood). As a rule, this version of chorea, as it progresses, is complicated by attacks of epilepsy and dementia.

  • Lesha-Naikhan's disease . This is a congenital pathology, which is caused by a hereditary deficiency of hypoxanthine-guanine phosphoribosyltransferase, which leads to serious disorders in the work of the nervous system and increased production of uric acid. The delay in motor development in the child is noticeable already in the first months of his life, then the stiffness of the limbs becomes more severe. The first facial grimaces, as a rule, occur already in the second year of life, there are also signs of defeat of the pyramidal tract and a delay in mental development. One of the threats of this pathology is that babies tend to do themselves physical harm by biting their fingers or sponges.

  • Benign (or non-progressive) hereditary chorea . This type of disease manifests itself in early childhood or even in infancy. The child has generalized hyperkinesis, which "subside" only during sleep. The main difference between the disease and Huntington's chorea is the following two points: the normal development of the intellect and the non-progressive course of the disease (on the contrary, in the case of benign chorea, it is even possible to reduce hyperkinesis at an older age).

  • Secondary forms. There are many, but the most common are chorea of ​​pregnant women and small chorea.

  • Chorea of ​​pregnant women, as a rule, appears in those women who in childhood have had a small chorea. This pathology is often manifested in primiparas. It manifests itself on the 2-5th month of pregnancy and can be repeated at subsequent "interesting" positions. Symptoms of the disease, as a rule, go away for a couple of months after childbirth or abortion.

  • Small chorea (name-synonym - chorea of ​​Sydenham). Often occurs within a couple of months after exacerbation of rheumatism or stentococcus infection. With mild forms, disinhibited movements with expressive gestures and exaggerated grimaces are noted. In more complex forms, the chorea hyperkinesis in the whole body deprives the patient of the ability to move normally, serve himself, communicate and even breathe. Muscular hypotension, emotional-affective disorders, "congealing" or "tonic" knee-jerk reflex are also observed.

  • The history of diseases - Chorea - St. Vitus' Dance

    Chorea and its treatment

    Therapeutic actions depend on the specific type of disease . Thus, with Huntington's chorea, there are effective agents that reduce the activity of dopaminergic brain systems: reserpine, haloperidol, phenothiazine derivatives, and the like. Tranquilizers and neuroleptics also help the patient cope with depression and internal discomfort. With a small chorea, you should go to a hospital. In addition to bed rest, antibiotics (peni- and novocellin), glucocorticoid hormones, salicylates, calcium preparations, cardiac drugs, multivitamins are prescribed. From fizioprotsedur actual conifer baths, electrosleep, galvanic collar for Shcherbak with calcium chloride.

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