Minor chorea (other name: Sydenham's chorea) in children is a neurological disease manifested by rheumatic infection.

This disease consists of an involuntary increase in motor activity (hyperkinesis).

It occurs as a result of damage to the parts of the brain responsible for coordinating movements and regulating muscle tone. The basis of the disease is inflammation of the subcortical elements of the brain.

The pathological substrate consists of inflammatory, vascular and degenerative changes nervous tissue.

In other words, this type of chorea can be considered encephalitis (inflammation of the brain) of a rheumatic nature. The heart is also affected by chorea minor.

Chorea minor is considered a childhood disease, since children usually get sick school age(5-16 years old), mostly girls. Perhaps this is due to the female hormonal levels. The disease often begins in late autumn and winter.

Causes

To date, the question of the nature of this disease still remains open. However, most doctors are inclined to consider the cause of this pathology to be a streptococcal infection.

This infection usually penetrates by airborne droplets into the upper respiratory tract, and it is this infection that, under favorable conditions, causes such widespread known disease throat like sore throat.

Trying to defeat the disease, the child’s body intensively produces antibodies to streptococcus.

In some cases, for reasons that are not fully understood, antibodies to the cerebral ganglia (the internal parts of the cerebral hemispheres lying immediately under the cortex) begin to be produced along with the latter.

Subsequently, a conflict begins between these antibodies and the ganglia.

It causes an inflammatory reaction under the cerebral cortex, resulting in hyperkinesis.

It happens that the cause of chorea in a child is congenital dysfunction basal ganglia(ganglion) of the brain, inherited.

Modern neurology has not yet fully elucidated the nature of this disease, but the main suspected cause of chorea minor, as well as childhood rheumatism, is considered to be a recent infectious disease of streptococcal nature.

Symptoms

Clinical symptoms of minor chorea become noticeable within 3-4 weeks after a sore throat.

The first signs are mental disorders: the child becomes distracted, uncollected, and capricious.

Movements become awkward, clumsy - everything falls out of his hands. Schoolchildren's academic performance drops; previously neat notebooks become dirty and sloppy.

The main signs of minor chorea appear - impaired coordination of movements, involuntary chaotic twitching of the limbs or parts of the face (hyperkinesis). Uncontrollable muscle contractions may involve the tongue, lips, larynx, diaphragm, or even the entire body.

At the beginning of the disease, hyperkinesis is hardly noticeable, often parents do not notice them or do not attach importance to them. Involuntary contractions facial muscles may be mistaken for grimacing or self-indulgence. Twitching intensifies when nervous tension or excitement, for example, when a child is called to the board to answer.

As the disease progresses, hyperkinesis becomes more noticeable and pronounced, with severe stressful situation the body of a sick child can be shaken by so-called “choreic storms”, in which involuntary muscle contractions occur simultaneously in all limbs and on the face. Sometimes violent movements, or, conversely, muscle weakness, develop only on one side of the body. It is characteristic that there are no involuntary twitches during sleep, but a sick child usually has difficulty falling asleep.

So, the signs of chorea minor:

• recently appeared antics, grimacing, sticking out the tongue;
• strange behavior (tearfulness, forgetfulness, sometimes complete indifference to the outside world);
• absent-minded, darting gaze, inability to concentrate on one thing;
• inability to calmly stay in one position (for example, during a lesson at school);
• difficulties with writing and drawing (the child cannot hold a pen or pencil, is unable to draw a straight line, stains notebooks);
• difficulty eating, walking, dressing, washing;
• decreased muscle tone - in some forms of the disease, hyperkinesis is almost invisible, but the muscles are so weak that the sick child becomes almost immobile;
• when hyperkinesis spreads to the tongue and larynx, uncontrollable screams are possible;
• previously clear, clear speech the child becomes slurred and confused; sometimes, with severe hyperkinesis of the larynx and tongue, speech may completely disappear.

In order to recognize minor chorea in time and begin treatment as soon as possible, parents should not ignore any of the above-described hyperkinesis at the beginning of their occurrence.

Diagnostics

A neurologist is involved in identifying and treating this pathology. First, it is determined in detail what diseases the child has had in the past, what medications he took, and whether any of his close relatives suffered from a similar illness.

The next stage will be to examine the child and conduct several diagnostic tests, during which it is possible to identify features characteristic of childhood chorea:

• a sick child is unable to stick out his tongue with his eyes closed;
• if you ask a child to stretch his arms forward, he will position his hands in a special way;
• when tapping the knee with a hammer, the leg is held in a straight position for some time due to hyperkinesis of the femoral muscle (Gordon's phenomenon);
• If you take a child with chorea minor by the armpits and lift him up, his head will “sink” into his shoulders (flabby shoulder syndrome).

Additionally, for more full picture diseases, the doctor should prescribe the following diagnostic tests:

• magnetic resonance imaging (MRI) of the brain;
• general blood test;
• electroencephalography;
• computed tomography of the brain;
• electrocardiogram;
• electromyography – study of biological capabilities skeletal muscles.

Treatment

Treatment acute stage the disease is carried out in a hospital setting.

A sick child needs bed rest and a calm environment.

Sleep promotes recovery, so doctors recommend treatment with drugs that calm the nervous system.

It is necessary to limit any physical activity child. Salicylates, pyramidon, calcium preparations, and vitamin complexes are widely used for the treatment of chorea minor. Treatment with certain pituitary hormones is possible.

To restore coordination of finger movements, it is recommended to involve a child who is already recovering in simple manual activities: sewing, knitting, drawing, cutting, modeling.

A recovering child should spend at least two hours a day on fresh air. It is important to feed him well - the children's diet should contain foods rich in vitamins and proteins (cottage cheese, milk, fish, eggs, lean meat) every day.

The main conditions for recovery are: more sleep, peace and positive emotions.

Procedures

In addition to drug therapy, for a speedy cure for minor chorea, physiotherapeutic procedures performed in a hospital are recommended for sick children.

In order to improve blood supply and metabolic processes in the brain, and provide an anti-inflammatory effect on the lesion, the following is prescribed:

1. Fresh or salty warm pine baths. 12-14 procedures for 10-12 minutes every day.
2. Aeroion therapy under voltage 25-35 kV, 10-12 procedures for 6-8 minutes every other day.
3. UHF of the fronto-occipital lobe of the brain. Every day for 13-14 minutes, a total of 15-18 procedures.
4. Electrosleep lasting 45 minutes every day, 20-25 procedures.
5. Electrophoresis of sodium salicylate (anti-inflammatory substance). The duration of exposure is 25-30 minutes. The course of treatment is 15-20 procedures every other day.
6. Calcium electrophoresis collar area, 12-14 sessions of 12-14 minutes every other day.
7. Ultraviolet irradiation of the collar area. 5-6 sessions every two days on the third.

Minor chorea lasts from 1 month to six months and with proper treatment ends with recovery. Relapses occur in approximately 30% of cases.

Protect your child from tonsillitis and rheumatism, and then minor chorea will bypass him. And if you do have a sore throat, treat it carefully and don’t let it happen. physical activity at least a month after recovery.

Video on the topic

The basis of this disease is rheumatic damage to the blood vessels of the brain. The subcortical nodes of the brain are predominantly affected. Chorea can be called a rheumatic breed. Very often the disease is combined with rheumatic lesions. Children are most often affected, with girls getting sick more often.

The beginning is not pronounced. The first symptom is an increase in motor activity (hyperkinesis): children spill the contents of plates and cups, drop the spoon from their hands, their gait is disrupted, their handwriting changes (letters become uneven and jumpy). The child often grimaces, his movements become somewhat pretentious and unnatural. Usually at this time, adults and teachers regard the child's behavior as a prank and often punish him.

As the process progresses, hyperkinesis intensifies, movements become uncoordinated and irregular. Facial features include frowning of the eyebrows, pulling of the mouth to one side, and twitching of the tongue. When the neck muscles are involved in the process, nodding, tilting or extension movements of the head appear. Gradually everyone is involved in the pathological process large groups muscles. Sometimes it comes to the so-called motor storm, which deprives the child of the ability to move and care for himself.

When the first signs of the disease appear, it is necessary to contact and undergo examination as soon as possible. The child is prescribed strict bed rest with additional hours of sleep (since prolonged hyperkinesis tires the child and deprives him of strength). The patient is given sedatives. Treatment of chorea should be aimed primarily at treating the underlying disease that caused it (encephalitis, etc.). (E.G. Uzhegov)

Additional information from the Great Soviet Encyclopedia

Chorea(from the Greek chor?ia - dancing) is a type of hyperkinesis, which is manifested by rapid twitching of the limbs, winking, smacking, etc. It occurs with organic damage to some subcortical parts of the brain. The most common form of chorea is minor chorea, or Sydenham's chorea, which usually develops in children and adolescents as one of the manifestations. In addition to hyperkinesis, it is characterized by a decrease in muscle tone, asthenic manifestations (impairment, tearfulness, irritability, etc.). The course of minor chorea is usually favorable, but relapses are possible. T.n. major chorea - hysterical choreiform twitching, observed in the Middle Ages as a mass phenomenon - is only of historical interest.

The so-called late chorea - Huntington's chorea (was described in 1872 by the American psychiatrist J. S. Huntington) - refers to hereditary degenerative diseases; is inherited in an autosomal dominant manner, most often manifests itself at the age of 35-40 years, and is characterized by a chronic progressive course. Muscle tone in some cases is reduced, in others it is increased (rigid form). The most important sign Huntington's chorea - mental disorders in the form of apathy, decreased memory and intelligence, unstable delusional ideas, hallucinations, etc. Deep dementia gradually develops. Along with damage to the subcortical regions, atrophy of the cerebral cortex is detected in Huntington's disease.

Used for the treatment of minor chorea antirheumatic (salicylates, etc.), sedatives and (diphenhydramine, suprastin, etc.) agents. For late chorea, aminazine, reserpine are prescribed, and for its rigid form, anticholinergic drugs (cyclodol, etc.), L-Dopa, midantan are prescribed. (V. A. Karlov)

Read more about chorea in the literature:

• Anosov N. N., Huntington's chorea, in the book: Multi-volume guide to neurology, vol. 7, L., 1960;
• Zucker M. B., Infectious diseases of the nervous system in children, M., 1963;
• Gittik L.S., Mala chorea, Kiev, 1965.

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Chorea is a disease of rheumatic etiology, characterized by movement disorders.

Damage to the rheumatic process of the body begins with the penetration of group A hemolytic streptococcus bacteria into the body. This microbe from foci of infection (for example, in chronic tonsillitis from the palatine tonsils) penetrates into bloodstream, distributed in various tissues. The pathogenesis of rheumatic lesions is based on pathology connective tissue. Damage to heart valves and joints may develop. Penetration of the pathogen into the central nervous system can provoke dysfunction of the cerebral cortex, cerebellum, basal ganglia (neurorheumatism). Large and small vessels of the brain are damaged with the development of vasculitis and thrombusculitis. As a result of the development of degenerative processes, coordination of movements and muscle tone are disrupted in these areas of the nervous system.

After the discovery of antibiotics, the incidence of chorea minor is up to 10% of all neurological diseases. The disease develops more often in girls than in boys, and mainly in the autumn-winter period. The duration of the disease is from 3 to 6 weeks. Short-term relapses may occur over a long period of time after the height of the disease (for example, during pregnancy). In rare cases, deaths have occurred due to pathological effects on the cardiovascular system.

Lesser chorea - Types

Depending on the picture clinical course highlight:

• Classic version of the course of minor chorea
• Atypical course of chorea:
  • erased, asymptomatic, sluggish form of the disease
  • paralytic minor chorea
  • pseudohysterical minor chorea

The course of the disease can be:

• Latent (hidden)
• Subacute
• Acute
• Recurrent

Minor chorea - Causes

Among the causes of the development of minor chorea and risk factors for this pathological process The following can be highlighted:

• Hereditary predisposition
• Female gender
• Age from 6 to 15 years
• Previous streptococcal infection (for example, sore throat)
• Asthenic physique
• Psychological trauma
• Increased excitability of the child's nervous system
• Frequent sore throats
• Chronic tonsillitis
• Respiratory diseases
• Rheumatism
• Decreased immunity
• Carious teeth

Lesser chorea - Symptoms

The development of minor chorea is accompanied by the following clinical signs:

• Twitching of arms and legs (trochaic hyperkinesis) on one side or symmetrical
• Twitching of facial muscles (grimacing, blinking, twitching of the corner of the mouth, wrinkling of the forehead)
• Muscle weakness
• Gait disturbance
• Shoulder Jerking
• Movement coordination disorder
• Head twitching
• Handwriting disorder
• Sleep disorders
• Seizures
• Speech impairment (lingual hyperkinesis)
• Difficulty swallowing (hyperkinesis of the laryngeal muscles)
• Intermittent breathing (diaphragmatic hyperkinesis)
• Emotional excitability
• Memory and attention disorders
• Increased fatigue, irritability
• Psychoses with auditory and visual hallucinations

Lesser chorea - Diagnosis in Israel

Diagnosis of minor chorea begins with collecting an anamnesis of the patient’s life and illness. The diagnosis is confirmed by a characteristic clinical picture in combination with certain research methods:

• Blood test– allows you to identify the content of markers of streptococcal infection in the body: antisteptolysin-O, rheumatoid factor, cyclic citrullinated peptide, C-reactive protein.
• Electromyography– a method that allows you to study the biopotentials of skeletal muscles. Upon registration electrical activity muscles with minor chorea, an elongation of potentials and asynchrony in their appearance are detected.
• Electroencephalogram– reveals diffuse slow-wave bioelectrical activity of the brain.
• , positron emission tomography– help identify focal changes in the brain.

Lesser chorea - Treatment in Israel

Israeli doctors have extensive experience in the treatment of chorea minor and can offer you the following treatment methods:

• Antibiotics penicillin series(bicillin)– used to combat streptococcal infections.
• Non-steroidal anti-inflammatory drugs (sulindac, ketorolac, derivatives salicylic acid) – reduce inflammatory syndrome in tissues.
• Glucocorticoids (prednisolone)– prevents the development of edema and inflammation in tissues, and an allergic reaction.
• Sedatives, tranquilizers (benzodiazepines, barbiturates)– relieve neuropsychic tension.
• Neuroleptics– drugs that inhibit the transmission of dopamine (one of the neurotransmitters). Haloperidol is used in combination with aminazine and reserpine, which block the transport of dopamine in nerve endings.
• Dopamine competitor drugs (dopegit)- when receiving them medicinal substance binds to dopamine receptors, which prevents the attachment of the endogenous mediator.
• Antihistamines (suprastin, diphenhydramine)– eliminate the allergic component of the disease.
• If there are risk factors (for example, chronic tonsillitis) preventive rinsing is indicated, and if it is ineffective– removal of tonsils.
• When stopping the acute stage of the process, a stay at the sanatorium resorts of the Dead Sea, famous for its healing and restorative effects, will have a positive effect on the patient’s body.

Chorea is a disease that requires special attention from a doctor when identifying and treating it.

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Sydenham's chorea (Sudenham, 1636) – neurorheumatism (see).

* * *
(by name English doctor Th. Sydenham, 1624–1689; synonyms - dance of St. Vitus, minor chorea, rheumatic chorea) - a manifestation of rheumatic encephalitis with damage to the cerebellum and its peduncles; in modern clinical practice is extremely rare. It occurs almost exclusively in childhood and adolescence (5–15 years), its appearance at an older age is regarded as vasculitis of the central nervous system (more often with systemic lupus erythematosus). Associated with the formation of antineuronal antibodies that interact with basal ganglia antigens. It usually occurs several months after an acute attack of rheumatism, so it is often not possible to identify other signs of rheumatism or streptococcal infection in patients. It manifests itself as bilateral or unilateral (hemichorrhea) hyperkinesis, occurring acutely or subacutely, with an increase over 2–4 weeks.

and involvement of the larynx and tongue, dysarthria and swallowing disorders develop. In mild cases, there may only be grimacing and mannered gestures (a consequence of the patient’s desire to give the appearance of purposeful movements to involuntary movements). In addition, muscle hypotonia is characteristic (with the so-called soft chorea, it “masks” chorea), decreased tendon reflexes, and a “freezing” knee reflex (prolonged choreic contraction of the quadriceps femoris muscle when its tendon is repeatedly tapped). Mental changes are often detected (affective lability, anxiety-depressive and obsessive states, decreased attention and memory), autonomic disorders(lability blood pressure, tachycardia). In most cases, hyperkinesis spontaneously regresses within 3–6 months. Relapses of the disease are possible, including during pregnancy, taking oral contraceptives, psychostimulants, levodopa, diphenine. In the long-term period, some patients who have had Sydenham's chorea exhibit slurred speech, awkwardness of movements, tremors, tics, asthenic, obsessive or anxiety-depressive disorders. Treatment: bed rest acute period, small doses of benzodiazepines or barbiturates, more severe cases neuroleptics are used valproic acid or carbamazepine. Glucocorticoids, plasmapheresis, and intravenous immunoglobulin are used. Those who have had Sydenham's chorea require prophylaxis with benzathine benzylpenicillin for 5 years.

T. Sydenham. Schedula monitoria de novae febris ingressu. Londini, 1686; p. 25–28.

Encyclopedic dictionary of psychology and pedagogy. 2013.

Notes: The presence of two major criteria or one major and two minor criteria in combination with documented evidence of previous infection with group A streptococci indicates a high likelihood of acute rheumatic fever. Special cases:

1. Isolated chorea - with the exclusion of other causes (including PANDAS*).

2. Late carditis - development of clinical and instrumental symptoms of valvulitis extended over time (more than 2 months) - with the exclusion of other causes.

3. Repeated acute rheumatic fever with or without chronic rheumatic heart disease.

* PANDAS is an acronym English words“Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections” (children's autoimmune neuropsychiatric disorders associated with streptococcal infection." This is a condition related in pathogenesis to rheumatic chorea, but differs from it in its normal neurological status. Clinical picture characterized only by behavioral disorders in the form of obsessive-compulsive disorder and (or) tic disorder.

Undoubtedly, to the serious achievements of science of the twentieth century. should include the development of prevention of acute rheumatic fever and its relapses. The basis primary prevention acute rheumatic fever are timely diagnosis and adequate therapy for active chronic infection pharynx (sore throat, pharyngitis). Taking into account global clinical experience, adapted to the conditions Russian healthcare recommendations for rational antimicrobial therapy of tonsillitis and pharyngitis.

Secondary prevention is aimed at preventing recurrent attacks and progression of the disease in people who have had an acute rheumatic fever, and involves regular administration of long-acting penicillin (penicillin benzathine). The use of this drug in the form of bicillin-5 made it possible to significantly (4-12 times) reduce the frequency of repeated rheumatic attacks and, consequently, increase the life expectancy of patients with RPS. At the same time, a number of authors pointed out the insufficient effectiveness of bicillin prophylaxis in 13-37% of patients. Joint research carried out at the Institute of Rheumatology of the Russian Academy of Medical Sciences and the State scientific center on antibiotics have shown that they are currently highly effective and safe medicine secondary prevention of acute rheumatic chorea is benzathine benzylpenicillin, prescribed at a dose of 2.4 million units intramuscularly every 3 weeks.

launched by domestic industry, prolonged dosage form penicillin - bicillin-5 - is currently not acceptable for secondary prevention of ARF, since it does not meet the pharmacokinetic requirements for preventive drugs. In the coming 21st century. Scientists' efforts will be focused on creating and improving a vaccine containing epitopes of M-proteins from “rheumatogenic” strains that do not cross-react with tissue antigens of the human body.

General information

So, what is minor chorea? Chorea minor is a rheumatic disease of a neurological nature, which is manifested by excessive motor activity of the patient’s limbs. This disease is known as rheumatic chorea and Sydenham's chorea, after the scientist who discovered this disease in 1686 and described its symptoms.

This disease manifests itself to a greater extent in children, however, there is evidence of the occurrence of the disease in adults.

Reasons

The main causative agent of the disease is group A hemological streptococcus. This streptococcus is well known to all parents, since it is precisely it that is to blame for the child’s sore throat or other infectious ailments of the upper respiratory tract(VDP).

It is believed that a child who is ill infectious disease VDP immediately goes into the risk group, as a potential patient diagnosed with chorea.

However, despite the fact that this reason is one of the main ones, there are a number of factors that can trigger the development of this disease, including:

• heredity;
• rheumatic diseases in the body;
• disruptions in the functioning of the endocrine system;
• the presence of carious formations on the teeth;
• decreased immunity;
• taking certain medications;
• cerebral palsy;
• insufficient blood supply to the brain

This is far from full list reasons that can provoke chorea. You should know that you are most susceptible this disease children aged 5 to 15 years due to hormonal changes occurring in their bodies. Girls especially suffer from the disease, since their concentration of hormonal surges is several times higher.

Symptoms

The main symptoms of minor chorea are the formation of hyperkinesis of varying intensity in the patient.

Hyperkinesis - no voluntary movements or muscle contractions

Since it is affected nervous system, then in addition to hyperkinesis, the following are diagnosed in a small patient: clinical manifestations diseases:

• uncontrolled movements of the limbs (especially evident during writing or drawing);
• grimacing;
• regular and uncontrollable scratching of the baby, the inability to sit quietly in one place, the need to touch something on the body, etc.;
• slurred speech (in especially severe cases it can manifest as complete loss of speech);
• shouting some words or sounds;
• moodiness;
• touchiness;
• anxiety;
• decreased muscle tone;
• when lifting the baby by the shoulders, the head seems to sink into the shoulders (a kind of pressing of the head into the neck occurs);
• inability to raise their arms up with their palms facing inward (the child raises them with their palms facing outward);
• inability to stick out tongue eyes closed;
• blueness of the feet and hands;
• cold extremities;
• low blood pressure.

In adults who are childhood Having suffered this disease, a heart defect can be diagnosed.

Diagnostics

This disease is diagnosed using an integrated approach to its study.

At the very beginning, the doctor will receive primary data about the patient’s condition, symptoms and thus compile an anamnesis.

Direct diagnostic measures include:

• clinical blood test;
• neurological tests (checking the body's response using neurological methods);
• electromyography;
• computed tomography;
• magnetic resonance imaging;
• electroencephalography.

An integrated approach to the study of the disease will allow you to make a timely and accurate diagnosis and prescribe effective treatment.

Treatment

Therapy for chorea minor consists, first of all, in eliminating the cause of the disease, which in most cases is an infectious disease.

The basis of treatment is antibiotic therapy. Penicillin and cephalosporin, as well as drugs based on them, are used as the main antibiotics for the treatment of Sydenham's chorea.

Naturally, as a maintenance therapy for the gastric microflora, the doctor prescribes a course of bifidobacterial drugs (Linex, Bakset). This therapy is especially necessary for young children, since their fragile bodies are not able to cope with such stomach disorders on their own.

In addition, for the treatment of minor chorea, sedative and tranquilizing drugs may be required, which are prescribed in case of negative reactions from the psycho-emotional state of the child.

In addition, in most cases, it is possible to prescribe anti-inflammatory drugs to reduce inflammation in the diseased body.

In especially severe cases, the patient is required to undergo bed rest, limiting the entry of bright light and loud sounds into the room.

The entire complex of treatment must be carried out under the supervision of a specialist - a neurologist. Any self-treatment, especially those prescribed based on the results of studying information via the Internet are strictly contraindicated.

Prognosis and prevention for chorea minor

Sydenham's chorea is not a fatal disease and, with proper treatment, goes away in 5-6 weeks.

Naturally, if a child is re-infected with streptococcal infection or rheumatism, a relapse is possible.

Among the most unpleasant complications, past illness can be attributed:

1. Heart defect.
2. Aortic insufficiency.
3. Mitrine stenosis.

Despite the fact that the disease is not considered fatal, there have been cases of death due to sudden disruptions of the cardiovascular system.

As preventive measures The following should be noted:

• timely treatment of infectious diseases and rheumatic diseases;
• complete and harmonious physical development baby;
• complete and balanced nutrition;
• strengthening the child's immunity.

So, chorea minor is not fatal, but unpleasant disease with the presence of complications, so timely contact with a specialist will avoid problems in the future for both you and your baby. Take care of your children and get treatment correctly!

CHAPTER 17. RHEUMATIC CHOREA (SYDENGAM'S CHOREA, MINOR CHOREA)

CHAPTER 17. RHEUMATIC CHOREA (SYDENGAM'S CHOREA, MINOR CHOREA)

Rheumatic chorea (Sydenham's chorea, minor chorea) is a rare post-infectious complication of tonsillitis (tonsillitis) or pharyngitis caused by group A β-hemolytic streptococcus, characterized by involuntary chaotic movements in the limbs and behavioral disorders.

Thomas Sydenham (1624-1689) - a talented English researcher and insightful doctor, British Hippocrates, as he was then called, for the first time in 1686 gave such an accurate description of chorea that his name remained forever associated with this form of the disease: “... in Mostly, St. Vitus's chorea occurs in children from 10 years to adolescence. Initially, there is a limp when walking, or rather dancing with dragging the leg like a jester; later similar movements appear in the hand on the same side; when this illness completely takes possession of him, he cannot remain in the same position for a single minute; the movements affect the torso or other parts of the body and change the posture and localization of the twitches... To drink from a cup, he makes a thousand gestures, like a juggler, before bringing it to his mouth properly. His hand sways from side to side, and finally he quickly pours the contents into his mouth and drinks greedily, as if he is trying to make others laugh.”

T. Sydenham did not correlate this disease with rheumatic fever, but described the main clinical symptoms diseases such as a variety of involuntary rapid and chaotic movements of the limbs, muscle weakness and emotional lability. Since then, this term - “St. Vitus’ dance” - has become widely used to refer to acute chorea, although this concept was known back in the 16th century, when T. Paracelsus (1493-1541) described "thorea naturalis" as an unusual choreiform disease (“dance frenzy”, “St. Vitus’s dance”), common in medieval Europe. In those days, this term was used for many nosological forms: hysteria caused by religious fanaticism, epileptic seizures, ergot poisoning, torsion dystonia. In 1894, W. Osler commented on this terminological confusion: “... it would be incorrect to say that Sydenham accidentally called the disease St. Vitus’s dance/trochea, but used this term in a new meaning; and this is not the only

There is a good example in medicine when we use a name for a disease whose original meaning has long been lost.”

Etiology and pathogenesis. The rheumatic origin of Sydenham's chorea was first suggested by M. Stol in 1780, and by the beginning of the 20th century. it has been irrefutably proven. Currently, the disease is very rare. Typically, rheumatic chorea manifests itself in childhood with a peak onset between 7-12 years. The disease most often affects girls over 10 years of age, which may be due to hormonal changes at this age. Typically, the first symptoms of minor chorea develop 2-7 months after a streptococcal infection.

Immunofluorescence analysis of blood serum in patients reveals an increase in titers of immunoglobulin G to group A β-hemolytic streptococcus. It is currently believed that the main pathogenetic mechanism for the development of the disease is the development of a cross-autoimmune response. In this case, cross-antibodies are formed to the membrane of group A β-hemolytic streptococcus and the cytoplasm of neurons of the subthalamic and caudate nuclei. However, with the disease, immunological parameters may be normal, which is associated with a long interval between the streptococcal infection and the appearance of neurological disorders (the phenomenon of molecular mimicry). Antiphospholipid antibodies are detected in 80% of patients with rheumatic chorea, but their role in the pathogenesis of the disease is still not fully understood.

Clinical manifestations. Minor chorea is usually combined with other clinical syndromes ARF (carditis, polyarthritis), however, in 5-7% of patients it may be the only manifestation of the disease.

Typically, the manifest symptoms of rheumatic chorea are behavioral disturbances (increased irritability, mood changes), awkwardness of movements and difficulty writing. “...A child with Sydenham's chorea will be punished three times before he is correctly diagnosed: once for restlessness, once for breaking dishes and once for making faces at his grandmother.” This statement by Wilson accurately illustrates the three main clinical features of chorea minor: spontaneous and involuntary movements, uncoordinated voluntary movements, and muscle weakness.

Involuntary movements are usually generalized, can rarely be asymmetrical and in 20% of cases are unilateral. Typically, hyperkinesis occurs in the facial muscles and distal sections limbs. At first they are barely noticeable and intensify only with waves.

neniya. As the disease develops, isolated sudden and short-lived involuntary movements become generalized. In the advanced stage of the disease, choreic hyperkinesis practically does not stop, disappearing during sleep and with drug sedation. In rare cases, the disease debuts with the development of severe generalized muscle hypotonia, in which the child cannot begin voluntary movements and the impression is of the development of flaccid paralysis, sometimes only one-sided. In this case, involuntary movements are very rare or completely absent. Speech disturbances are observed in 15-40% of cases; in severe cases, swallowing disturbances may occur. Very rarely, the first symptoms of the disease may be seizures, cerebellar, pyramidal disorders and disc edema. optic nerves. Carditis develops in 75% of cases.

With minor chorea, deep tendon reflexes are reduced or torpid. There are no pathological reflexes.

At neurological examination There are 4 characteristic symptoms:

1) Gordon II reflex (when the knee reflex is evoked, the lower leg remains in the extension position for several seconds, caused by contraction of the quadriceps femoris muscle);

2) a symptom of a choreic hand - a pathological position of the hand, in which it is slightly bent at the wrist joint, and the fingers are in a position of hyperextension in the metacarpophalangeal joints and extended or slightly bent in the interphalangeal joints;

3) “chameleon tongue” - the inability to stick out your tongue with your eyes closed (when you try to stick out your tongue, it immediately returns to your mouth);

4) Cherny's symptom - retraction abdominal wall and rise of the diaphragm during inspiration.

The duration of rheumatic chorea is from 1 month to 2 years. The disease can recur during exacerbation of chronic streptococcal infection, but if no symptoms of the disease are observed during the first two years, chorea is unlikely to occur. The prognosis of the disease is usually favorable. However, emotional instability or minimal neurological symptoms such as awkward movements and tics may persist for several months. Girls who have had chorea remain at high risk of its occurrence during pregnancy and when taking contraceptives, which may be associated with increased dopaminergic sensitivity.

Diagnostics.When recognizing acute rheumatic fever, the syndromic principle developed by the domestic pediatrician A.A. is used. Kisel in 1940. He identified 5 main diagnostic criteria: migratory polyarthritis, carditis, chorea, annular erythema, rheumatic nodules, drawing attention to the diagnostic significance of their combination. In 1944, American cardiologist T.D. Jones classified the indicated pentad of syndromes as “large” diagnostic criteria, highlighting along with them “small” clinical and laboratory parameters. Subsequently, the Jones scheme was repeatedly modified by the American Heart Association (AHA) and became widespread.

In table 26 shows a diagram of diagnostic criteria Kisel-Jones taking into account the latest revision of the AKA (1992) and modifications proposed by the Association of Rheumatologists of Russia (ARR) in 2003. MRI of the brain visualizes both bilateral and unilateral increases in signal intensity in T2-weighted images in the region of the caudate nuclei and putamen . However, changes on CT/MRI of the brain are not pathognomonic for this disease. Positron emission tomography in the active stage reveals an increase in glucose metabolism in the thalamus and striatum, which is reversible.

EEG reveals nonspecific changes.

Differential diagnosis carried out with PANDAS syndrome, viral encephalitis, dysmetabolic encephalopathies, hereditary neurodegenerative diseases that occur with predominant damage to the subcortical structures of the brain.

Prevention and treatment. It is recommended to prescribe bed rest and protective rest. Thus, in the acute polysyndromic course of acute rheumatic fever or with the development of pancarditis, glucocorticosteroids are indicated - prednisolone or methylprednisolone (0.6-0.8 mg/kg/day) for 10-14 days, rarely longer, under the control of clinical and instrumental data , including dynamic echocardiography monitoring. With positive dynamics, they begin to decline daily dose glucocorticosteroids at 2.5 mg per week, then transferred to non-steroidal anti-inflammatory drugs in age-related doses. Even in cases where there is no clear immunological evidence of the disease and culture from the nasopharynx for streptococcal flora did not give positive result, it is necessary to conduct a course

antibacterial therapy. Prescribe benzylpenicillin (potassium and sodium salt) 150,000 units 4-5 times a day intramuscularly or orally at a dose of 200 to 250 mg 4 times a day for 10-14 days.

Table 26.Kisel-Jones criteria used to diagnose acute

rheumatic fever

Large criteria	Small criteria		Evidence of previous A-streptococcal infection
Carditis Polyarthritis Chorea Ring-shaped erythema Subcutaneous rheumatic nodules	Clinical	Arthralgia Fever	Positive A-streptococcal throat culture or positive rapid A-streptococcal antigen test. Increased or increasing titers of anti-streptococcal antibodies (ASL-O, anti-DNAase B)
	Laboratory	Elevated acute phase reactants: ESR, C-reactive protein
	Instrumental	Prolongation of the PR interval on the ECG Signs of mitral and/or aortic regurgitation on Doppler echocardiography

Notes: The presence of two major criteria or one major and two minor criteria in combination with documented evidence of previous infection with group A streptococci indicates a high likelihood of acute rheumatic fever. Special cases:

1. Isolated chorea - with the exclusion of other causes (including PANDAS*).

2. Late carditis - development of clinical and instrumental symptoms of valvulitis extended over time (more than 2 months) - with the exclusion of other causes.

3. Repeated acute rheumatic fever with or without chronic rheumatic heart disease.

* PANDAS is an abbreviation of the English words “Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections” (children’s autoimmune neuropsychiatric disorders associated with streptococcal infection.” This condition is related in pathogenesis to rheumatic chorea, but differs from it in its normal neurological status. The clinical picture is characterized only behavioral disorders in the form of obsessive-compulsive disorder and (or) tic disorder.

Undoubtedly, to the serious achievements of science of the twentieth century. should include the development of prevention of acute rheumatic fever and its relapses. The basis of primary prevention of acute rheumatic fever is timely diagnosis and adequate treatment of active chronic infection of the pharynx (sore throat, pharyngitis). Taking into account global clinical experience, recommendations for rational antimicrobial therapy of tonsillitis and pharyngitis have been developed, adapted to the conditions of Russian healthcare.

Secondary prevention is aimed at preventing recurrent attacks and progression of the disease in people who have had acute rheumatic fever, and involves regular administration of long-acting penicillin (benzathine penicillin). The use of this drug in the form of bicillin-5 made it possible to significantly (4-12 times) reduce the frequency of repeated rheumatic attacks and, consequently, increase the life expectancy of patients with RPS. At the same time, a number of authors pointed out the insufficient effectiveness of bicillin prophylaxis in 13-37% of patients. Joint studies carried out at the Institute of Rheumatology of the Russian Academy of Medical Sciences and the State Research Center for Antibiotics have shown that benzathine benzylpenicillin, prescribed at a dose of 2.4 million units intramuscularly every 3 weeks, is currently a highly effective and safe drug for the secondary prevention of acute rheumatic chorea. The long-acting dosage form of penicillin, bicillin-5, produced by the domestic industry, is currently not acceptable for secondary prevention of ARF, since it does not meet the pharmacokinetic requirements for preventive drugs. In the coming 21st century. Scientists' efforts will be focused on creating and improving a vaccine containing epitopes of M-proteins from “rheumatogenic” strains that do not cross-react with tissue antigens of the human body.