Chorea

Introduction to small chorea Small chorea, also known as rheumatoid chorea, is an encephalopathy of unknown cause. Often one of the manifestations of acute rheumatism. The disease is more common in children aged 5 to 15 years, many sub-acute onset, but also sudden onset due to emotional agitation. It is characterized by extremely fast irregular beats and meaningless dance-like movements, decreased muscle tone and muscle weakness, and often accompanied by psychological disorders. The disease can be self-healing, but recurrence is not uncommon. In addition to symptomatic treatment, modern medicine has no special treatment. basic knowledge The proportion of illness: 0.03% Susceptible people: no specific population Mode of infection: non-infectious Complications: mental disorders

Cause of small chorea

( 1) Causes of the disease

The disease is associated with type A hemolytic streptococcal infection, about 30% of cases occur after rheumatic fever or multiple arthritis 2 to 3 months, usually no recent history of sore throat or fever, some patients swallow swab culture A Positive for hemolytic streptococcus; serum can detect anti-neuronal antibodies, react with neuronal antigens such as caudate nucleus and subthalamic nucleus; antibody titer is related to the outcome of this disease, suggesting that it may be related to autoimmune response. The disease occurs in adolescence, more women, patients with pregnancy or oral contraceptives can relapse, suggesting that it is related to endocrine changes.

(two) pathogenesis

It is generally believed that susceptible children are infected with group A beta-hemolytic streptococcus, and the corresponding antibodies are produced. Such antibodies incorrectly recognize the caudate nucleus, the antigen of the subthalamic nucleus neurons, causing an inflammatory reaction and causing disease.

Uncomplicated acute chorea rarely dies, so there are few pathological data, but most authors believe that the main pathological changes of this disease are scattered in the cerebral cortex, basal ganglia, substantia nigra, subthalamic nucleus and cerebellar dentate nucleus. Arteritis and neuronal degeneration, even spotted hemorrhage can be seen, sometimes the brain tissue can present a small embolic small infarction, the pia mater can have mild inflammatory changes, and a small amount of lymphocyte infiltration around the blood vessels.

Evidence of rheumatic heart disease can be found in 90% of cases of autopsy.

Small dance disease prevention

Small chorea is a common manifestation of rheumatic fever in the brain. About 1/4 of patients have developed rheumatic fever such as joint pain, frequent sore throat, skin erythema, rheumatic heart disease, about 1/2 in or after the disease. A variety of rheumatic fever phenomena occur, so prevention and treatment of rheumatic fever is the key. The disease is slow due to slow onset and early symptoms are not obvious. In children, it can be manifested as distracted, restless, declining academic performance, awkward body movements, etc. Teachers are misdiagnosed as having neuroticism or naughtiness, so they should be noticed.

Small chorea complications Complications

Systemic symptoms can be mild or completely absent. There is no fever at the beginning of the disease, but in the later stages, symptoms such as fever, pale skin and low hemoglobin anemia may occur. People with rheumatic heart disease may have heart enlargement or murmur, and may have other manifestations of acute rheumatism, such as fever, arthritis, tonsillitis, subcutaneous nodules, and the like. Children often have certain psychiatric symptoms such as anxiety, depression, emotional instability, irritation, decreased concentration, and paranoid-forced behavior.

Symptoms of small chorea common symptoms, attention deficit, decreased muscle tone, irritability, ataxia, involuntary movement, joint pain, irregular breathing

1. About 2/3 of the patients are children aged 5 to 15 years old, and there are more women. The ratio of male to female is about 1:1, 5 to 1:3, 2. There are often respiratory infections, pharyngitis and other group A hemolysis. History of streptococcal infection, most of which are subacute or insidious onset, a few acute onset, early symptoms are often not obvious, not easy to be detected, manifested as children are more restless than usual, easy to be excited, distracted, academic performance decline The limbs are clumsy, the writing is skewed, the objects in the hands are often lost and the gait is unstable. At this time, parents or teachers can often misunderstand that the child is neurotic or by naughty, and the symptoms are getting worse. After a certain period of time, they appear. Dance-like movements and changes in muscle tone.

2, dance-like movements can be acute or insidious, often bilateral, about 20% of patients with lateral or local limitations, if limited to one side, called hemi-lateral chorea, dance-like movements are obvious, showing eyebrows , pouting tongues and grimacing; limbs appear a very fast, irregular, pulsating and meaningless involuntary movements, unlike those who are habitual or mentally sturdy, the upper limbs alternately stretch Straight, flexion and adduction, lower limbs gait bumps, walking and shaking, easy to fall, the trunk shows the spine constantly bending, stretching or twisting, breathing can be irregular, the tongue is difficult to maintain, the tongue keeps twisting, soft or Other involuntary movements of the pharyngeal muscles can cause dysphonia and dysphagia, emotional stress, increased symptoms during exercise and speech, lessening when quiet, disappearing during sleep, often exacerbated within 2 to 4 weeks, within 3 to 6 months Relieve on your own.

3, the patient's muscle tension and muscle weakness (limb weakness), and dance-like movements, ataxia constitutes a small chorea triad, the patient's arm over the head when the palm pronation (pronator muscle sign), when the arm stretched forward Because the tension is too low, the wrist is flexed, and the metacarpophalangeal joint is overextended. It is called the choreic hand. It can be accompanied by a finger-like piano-like dance. If the patient holds the examiner's 2nd and 3rd fingers, the patient can feel the patient. When the hand is tight, it is called "milkmaid grip" or wax-waning sign. The knee reflex is weakened or disappeared. The variant is a small chorea or a limited chorea. A small number of patients have hypothyroidism or paralysis due to pyramidal tract damage, called paralytic chorea.

4, can appear insomnia, restlessness, restlessness, insanity, hallucinations, delusions and other mental symptoms, called manic dance disease, surrounded by noisy sounds or strong light stimulation can make patients' commotion and dance-like movements significantly worse, some cases Psychiatric symptoms are as obvious as physical symptoms, resulting in mental illness, elimination with dance-like movements, and rapid relief of mental symptoms.

5, about 30% of cases may have rheumatic myocarditis, mitral regurgitation or aortic valve insufficiency, etc., may have other symptoms of rheumatic fever such as fever, rheumatoid arthritis and subcutaneous nodules, etc., the disease can be self-healing, However, recurrence is not uncommon. It has been reported that children with chorea patients have central retinal artery infarction. Most authors believe that this group of patients with recessive valvular disease caused by embolization of retinal artery, and another may be local vasculitis. And cause thrombosis.

Small chorea examination

Laboratory inspection

1. In typical children, peripheral blood leukocytes increased, erythrocyte sedimentation rate increased, C-reactive protein increased, anti-streptolysin "O" titer increased, throat swab culture detected type A hemolytic streptococcus, but no complications of dance Patients with blood, urine, erythrocyte sedimentation rate and C-reactive protein may also be normal, and may have eosinophilia.

2, cerebrospinal fluid examination: very few abnormalities, but there are reports of small chorea patients with increased number of mild cells in the cerebrospinal fluid.

Film degree exam

1. EEG examination: 55%75% of children with chorea are abnormal, but they are very mild. The incidence of abnormal EEG is the highest at the peak of the disease. After the clinical symptoms are recovered, the EEG is gradually recovered. This abnormal change is not specific, including high diffuse slow waves in the occipital region, decreased alpha rhythm, focal or epileptic seizures, and occasional 14 Hz or 6 Hz normal phase spikes.

2, 29% ~ 85% of patients with CT showed a low density lesion and edema in the caudate nucleus, MRI showed caudate nucleus, shell nucleus, T2W signal of globus pallidus enhanced, can be resolved when clinical improvement, SPECT can show caudate nucleus, In particular, the cerebral blood flow perfusion of the nucleus was reduced, and PET showed excessive glucose metabolism in the striatum and returned to normal as the symptoms subsided.

Diagnosis of small chorea

diagnosis

According to the age of onset, typical dance-like movements, decreased muscle tone, decreased muscle strength and other symptoms, diagnosis is not difficult, such as other manifestations of acute rheumatism (arthritis, tonsillitis, heart disease, increased ESR, etc.) The diagnosis is more certain.

There are 25% to 30% of small chorea patients, there is no other evidence of rheumatic fever, and there are no other rare causes of chorea. These patients are still rheumatoid chorea, but the dance-like action is rheumatic fever. The first symptoms are only present.

Differential diagnosis

Must pay attention to the identification of other causes of chorea and similar symptoms:

1, habitual sputum: also known as habitual movements, more common in children, no typical symptoms of rheumatism, characterized by action stereotypes, limited to the same muscle or muscle group, no muscle strength, abnormal muscle tone and ataxia, etc. .

2, congenital chorea: dance-like action can be used as a manifestation of cerebral palsy, more than 2 years before the onset, minor dance disease early, often accompanied by mental retardation, tremors and spasticity.

3, Tourette syndrome (Tourette syndrome): seen in children, rapid performance, stereotypes of repeated irregular multiple muscle twitching, often involving the head and face, neck muscles and throat muscles, as well as strange or spit.

4, Huntington's disease: more common in middle-aged, in addition to dance movements, often have a history of genetics and dementia, a small number of childhood onset with muscle rigidity.

5, torsion : common in childhood, sometimes reversed sputum movements can be mistaken for dance-like movements, childhood torsion often persists, no self-limiting, muscle tension increased when the limbs are twisted, normal when stopped.

6, hepatolenticular degeneration: more in the onset of adolescents, can also show a dance-like involuntary movement, but the onset is slow, progressive aggravation, copper metabolism disorders and family genetic history can be identified.

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