Chorea is ... Chorea in children. Rheumatic chorea

Chorea is a pathology of the nervous system. It belongs to the forms of hyperkinesis. The disease is accompanied by problems with subcortical nodes of the brain. Identify it can be on the basis of characteristic signs: episodic, aimless, chaotic twitching in the hands and feet, sometimes in the trunk. In the later stages, there are mental and intellectual disorders, coordination problems and self-care. In children, chorea is often taken as the usual increased activity. In fact, with the first manifestations of pathology, you need to seek qualified help.

The term "chorea"

The term is used in two cases. It can denote independent diseases that belong to this group (for example, small chorea or Huntington's chorea). It is also used to describe some of the syndromes that occur in a variety of diseases. The very word "chorea" is taken from the Greek language, where it denotes a certain kind of dance. This explains the first symptoms of the disease, which are manifested in steep, uncoordinated, jerky, rapid movements of the trunk and extremities. The behavior of the patient is really like an involuntary, disorderly dance. Once upon a time, chorea was called "the dance of St. Witt." This name is associated with the ancient belief about St. Witte, who actively spread Christianity, for which he was tortured by Roman soldiers. After his death, the legend became popular, that if someone performs an active dance near his grave on the day of his birth, he will receive a charge of vivacity and energy for a whole year.

Causes

The causes of the development of the disease can have a different origin, but most often the chorea is caused by hereditary factors or genetic disorders.

Less common is chorea caused by such infectious diseases as tuberculous meningitis, viral encephalitis, neurosyphilis, borreliosis, whooping cough. The occurrence of chorea can also be associated with metabolic disorders (if there is Fabry's disease, hyperglycemia, hyperthyroidism or Wilson-Konovalov's disease), intoxications (poisoning with oral contraceptives, mercury, lithium, digoxin, neuroleptics, levodopa), autoimmune pathologies (such as antiphospholipid syndrome , Systemic lupus erythematosus, multiple sclerosis). Among the causes that trigger the onset of the disease, doctors also call damage to the structure of the brain. This includes tumor formation, hypoxic encephalopathy, craniocerebral trauma, strokes.

General features

Chorea is a disease that manifests itself in random, chaotic, short-term bodily movements. In some cases, these movements can be taken as normal, adequate, characteristic for a healthy person, with something alarmed. In others, they resemble an emotional, uncontrollable dance. Jerking jerks can be one-or two-sided, but they are deprived at least of some kind of synchronism.

Depending on the type of pathology detected, the manifestations of chorea are different. If a person has slightly expressed choreic hyperkinesis, then his motor behavior is marked by a slight anxiety, accompanied by motor disinhibition, increased emotionality, certain grimaces, fussy movements, inadequate gestures.

Clearly expressed forms of chorea are manifested in the form of peculiar movements of the "devil on the thread." The pronounced form of the trochaic hyperkinesis distorts the movements in walking, speech and facial expressions. The patient's walk is not only strange, but also somewhat "clownish". Chorea, the symptoms of which are extremely difficult, do not allow any movement at all. Patients with a severe form of pathology are dependent on their environment, because they can not move and serve themselves in the home fully.

Houteon Huntington

This pathology in most cases occurs at the age of 35 to 45 years. Chorea, the symptoms of which, in addition to typical hyperkinesia, carry in themselves more personality disorders and dementia (loss of intelligence), develops gradually, sometimes it is even difficult to trace the moment when it began to manifest. The first violent movements, as a rule, occur on the face. They can be confused with random motor automatisms (this is a tongue poking out, frowning, licking lips, opening the mouth). Progression of the disease manifests itself in the development of hyperkinesis in the field of the trunk and extremities. Heavy form is characterized by impaired speech, memory, swallowing processes, self-service level and ends with dementia. The starting point for Huntington's chorea is mental disorders in the form of hallucinatory-paranoid, affective and behavioral disorders.

Neuroacanthocytosis

Patients with neuroacanthocytosis suffer not only in choreic hyperkinesis, but also with acanthocytosis (the shape of erythrocytes changes). A distinctive feature in this pathology is atrophy (weakness in the muscles of the upper and lower extremities). Among other characteristics of the disease are: oral hyperkinesis with chewing movements, twitching of the lips, protrusion of the tongue and other grimaces. With neuroacanthocytosis, the patient involuntarily bites the tongue, lips and inner surface of the cheeks to the blood. The complicating factors in this disease are dementia and epileptic seizures.

Lesha-Naikhan's disease

Congenital diseases of the nervous system prevent a person from living a full life. Such pathologies include Lesha-Naikhan's disease. The main cause of the development of pathology is the hereditary deficiency of hypoxanthine-guanine phosphoribosyltransferase. This chorea in children is accompanied by severe disorders in the functioning of the nervous system. Also, pathology provokes an increased production of uric acid.

From the first months of life, the child has a delay in development. Rigidity of the limbs is evident from the third month. Since the second year of life, the baby has facial grimaces, which are accompanied by signs of lesions of the pyramidal tract and a delay in mental development. A kid can do himself harm physically by biting his lips or fingers.

Benign chorea

Benign chorea in children manifests itself in infancy or early childhood. Pathology refers to hereditary diseases. Generalized hyperkinesis in this disease recedes only when the child is asleep. From Huntington's chorea, this type of pathology is characterized by non-progressive currents and normal development of intelligence. At an older age, with benign chorea, it is possible to reduce hyperkinesis if you contact a medical institution on time.

Secondary forms of chorea

The most common secondary forms of pathology are two: small chorea and chorea of pregnant women.

The first is called Sidenham's chorea. To provoke its occurrence may exacerbate streptococcal infection or rheumatism. The mild form is characterized by exaggerated grimaces, expressive gestures, disinhibited movements. More complex forms of choreic hyperkinesis are manifested in the fact that it is difficult for the patient to move around, speak normally, or even breathe. When the disease also occurs, "tonic" knee and "congealing" reflexes, emotional and affective disorders, muscle hypertension.

Pregnant chorea is a disease that returns from childhood. That is, those women who faced childhood with small chorea fall into the risk zone. The pathology can appear during the first pregnancy for 2-5 months. With the subsequent flow of the "interesting" position, chorea can also occur. Treatment in such cases is rare, since the disease disappears by itself after the termination of pregnancy or after childbirth.

Chorea rheumatism

The onset of the disease occurs against a background of acute rheumatism or endocarditis, which is accompanied by damage to the heart valves. Many cases of the disease are not associated with this factor, and the disease manifests itself.

Rheumatic chorea most often affects children aged 6 to 15 years. In girls, pathology is more common. At the first stage, the disease can not cause concern, because the symptoms are limited to grimaces, and parents take it as a joke. The process continues with small involuntary movements of limbs, starting from the tips of the fingers and spreading gradually throughout the body. A week later, there comes a period of manifestation, when the child's movements become swift and sweeping. This state prevents the baby from continuing to function normally, that is, eating, walking, writing.

Rheumatic chorea distorts the child's face with thousands of grimaces. Severe forms of the disease prevent the patient from standing on his feet, swallowing, talking, while the body is constantly moving. If chorea is diagnosed, treatment should be done carefully and urgently.

Diagnostics

Suspicions of chorea arise from the strange behavior of a person. At a preliminary examination, the doctor should conduct a conversation with the patient or with his relatives. In particular, it is important to find out if someone else was sick in the family with this disease, how long the symptoms of chorea appeared, whether the patient took medications, whether it was sick with inflammatory ailments. Further the expert should give an estimation to involuntary movements. It is also necessary to diagnose and describe diseases that may accompany more often, cause serious intellectual deviations. Symptoms of such diseases do not always manifest. Sometimes, in order to detect a similar pathology at an early stage, a whole complex of examinations is carried out. A blood test will show the level of copper in the blood and the presence or absence of signs of inflammatory reactions. In addition, computer tomography, magnetic resonance imaging, and electroencephalography are performed.

Treatment

Chorea is a pathology that does not allow the patient to lead a habitual full-fledged lifestyle, so her treatment should be timely and comprehensive. Tactics of treatment directly depends on the causes of the disease. For example, with Wilson-Konovalov disease, the patient is prescribed a diet with a minimum content of copper and drugs whose action is aimed at reducing its absorption.

In the case of Huntington's chorea, neuroleptics and benzodiazepine tranquilizers are prescribed. If the chorea is caused by chronic insufficiency of the blood supply to the brain, then drugs that lower the level of blood pressure are needed. When vasculitis is hormonal treatment. If there are very generalized symptoms, treatment should be comprehensive.

The intensity of involuntary movements can be reduced by surgical intervention, when the ventrolateral nuclei of the thalamus are destroyed. With horee, you need to take medications that improve the work and nutrition of the brain, as well as vitamins of group B.