Chorea : Causes, Symptoms, Diagnosis & Treatment

• General Characteristics
• Why Does Chorea Occur?
• Hereditary Diseases
• Acquired Chorea
• Diagnostics
• Treatment

Chorea is a type of hyperkinesis in which involuntary movements are observed, resembling normal ones, but differing from them in irregularity, disorder and randomness. It is detected in Huntington's disease, neuroacanthocytosis, Lesch-Nychen syndrome, spinocerebellar ataxia, and other hereditary pathologies. It briefly occurs in childhood with rheumatic diseases and lesions of the upper respiratory tract. The cause is established according to the anamnesis, neurological examination, additional studies. Antipsychotics are used to eliminate chorea. Surgical treatment is ineffective.

general characteristics

Chorea comes from the Greek word for a type of dance. In the literature, the symptom is also known as the "dance of St. Vitus." The reason for such names are the features of hyperkinesis, often reminiscent of dance movements. Pathology varies significantly in severity. In mild cases, it looks like excessive fussiness, restlessness and some inadequacy of gestures.

With an average degree, motor acts are pretentious, strange, the gait resembles a "clown's" one. In especially severe cases, involuntary motor activity does not allow standing and moving independently, limits or makes self-care impossible. Choreic movements of the facial muscles, tongue, muscles of the larynx and pharynx can make it difficult to eat and cause speech disorders.

Why does chorea occur?

hereditary diseases

Chorea is characteristic of a number of hereditary pathologies with degeneration of nerve cells or metabolic disorders, accompanied by the deposition of certain substances in cerebral tissues. Found in the following diseases:

• Chorea of ​​Huntington. The first symptoms can appear at any age, but, as a rule, the onset occurs at 35-4 years of age. At first, hyperkinesias are more often noted in the face area, they are licking of the lips, sticking out the tongue, opening the mouth, frowning. The chorea then spreads to the arms, later becoming generalized, disturbing standing and walking. dementia develops.
• Neuroacanthocytosis. The main signs are chorea and acanthocytosis (change in the shape of red blood cells). The debut falls on 30-4 years, accompanied by oral hyperkinesis. Then the muscles of the trunk and limbs are involved in the process, a generalized chorea occurs in combination with tics and freezing in elaborate postures. Subsequently, epileptic seizures, secondary parkinsonism are noted.
• Lesch-Nyhan syndrome. It is provoked by genetically determined disorders of purine metabolism and the accumulation of uric acid in the body. diagnosed in boys. The lag in the formation of motor acts is observed already at the 3rd month of life. By the first or second year, generalized hyperkinesis develops. The chorea is gradually aggravated, loss of ability to the purposeful movements is possible. The clinical picture resembles cerebral palsy. Typical auto aggression.
• Spinocerebellar ataxia. Chorea occurs in the most common types 1 and 2 of the disease. The average age of manifestation is 18-3 years. Clumsiness of movements is detected, gradually transforming into hand trembling, gait disturbances, handwriting changes and hyperkinesis. Ophthalmoplegia, cerebellar ataxia, secondary parkinsonism are formed. In some cases, atrophy of the optic nerve is detected.
• Wilson-Konovalov disease. The first symptoms appear in childhood or adolescence. Even before the appearance of neurological disorders in many patients, dyspepsia and deterioration of liver function are determined. Extrapyramidal symptoms include chorea, muscle rigidity, and psychiatric disturbances. Kaiser-Fleischer rings are visualized on the cornea, yellowish skin tone, increased bleeding, hypochromic anemia are possible.

Chorea

Acquired chorea

The most common disease accompanied by this type of hyperkinesis is chorea. Pathology often develops in girls aged 10-12 years. Streptococcal infection (tonsillitis, pharyngitis, tonsillitis) acts as a provoking factor. Children often suffer from rheumatism. Attacks of choreic movements are replaced by episodes of fading, and then by normal motor activity.

There is a decrease in muscle tone, emotional lability, tearfulness, irritability. Symptoms, on average, persist for 3 months, sometimes up to 6 months or up to a year. Relapses are possible. Other causes of acquired chorea include:

• Infectious diseases: viral encephalitis, tuberculous meningitis, neurosyphilis, neuroAIDS, whooping cough, borreliosis.
• Autoimmune pathologies: antiphospholipid syndrome, SLE, reaction to immunization.
• Metabolic disorders: hyperthyroidism, phenylketonuria, homocystinuria, mucopolysaccharidoses, frequent episodes of hyperglycemia and hypoglycemia in diabetes mellitus.
• Structural changes: strokes, brain tumors, encephalopathy.
• Intoxication: mercury poisoning, taking antipsychotics, oral contraceptives, digoxin, levodopa, lithium.

Diagnostics

Diagnostic measures are carried out by a neurologist. Depending on the etiology of the symptom, rheumatologists, infectious disease specialists, endocrinologists and other specialists are involved in the examination of patients with acquired chorea, depending on the etiology of the symptom. If you suspect a hereditary disease, you may need to consult a geneticist. If the clinical picture of the disease includes mental disorders, an examination by a psychiatrist is indicated.

During the survey, the moment of the first appearance of chorea, the change in hyperkinesis over time, and other complaints are determined. During the examination, the following procedures are carried out:

• Neurological examination. The doctor evaluates the severity and prevalence of chorea. It reveals paresis, dystonia, sensitivity disorders, cerebellar ataxia, manifestations of secondary parkinsonism, and other disorders that indicate the nature of the disease.
• Neuroimaging. According to CT or MRI of the brain with Huntington's chorea, atrophy of the heads of the caudate nuclei is detected, with neuroacanthocytosis - nonspecific atrophy of the striatum and caudate nucleus, with spinocerebellar ataxias and Wilson's disease - areas of neurodegeneration and demyelination in the region of the cerebellar vermis, cerebral hemispheres and basal nuclei.
• Other visualization methods. On CT of the brain, the same changes are seen as on MRI. According to PET of the brain in case of minor chorea in the active stage, an increase in glucose metabolism in the thalamus and striatum is confirmed, with neuroacanthocytosis - a decrease in the number of dopamine receptors, activation of metabolic processes in the striatum.
• Electrophysiological methods. In patients with neuroacanthocytosis, an increase in epileptiform activity is determined during electroencephalography, and axonal polyneuropathy is detected during electroneuromyography. In patients with minor chorea, diffuse slow waves are detected on the EEG.
• Laboratory tests. Hereditary diseases are verified using molecular genetic tests. With neuroacanthocytosis, erythrocytes with spike-like outgrowths are visualized in a blood smear. Wilson's disease is characterized by a decrease in the level of ceruloplasmin in the biochemical blood test and an increase in the amount of copper in the daily urine. The suspicion of chorea is an indication for the determination of rheumatoid factor, C-reactive protein and antistreptolysin-O.

Patients with neuroacanthocytosis and spinocerebellar ataxia are recommended to consult an ophthalmologist to identify and assess the severity of visual impairment. In pathologies accompanied by cognitive decline and psychoemotional disorders (Huntington's chorea, neuroacanthocytosis), neuropsychological testing is performed.

Neurological examination

Treatment

To eliminate or reduce the severity of chorea, adults with neuroacanthocytosis, spinocerebellar ataxia, Huntington's disease, Wilson's disease with a predominance of hyperkinesia are prescribed antipsychotics. With small chorea, along with neuroleptics, hormones, antibacterial, anti-inflammatory and anticonvulsant drugs are used.

Etiopathogenetic therapy for most hereditary diseases has not been developed. In Wilson's disease, thiol agents are recommended. Patients with neuroacanthocytosis with the development of epileptic seizures are shown benzodiazepines, with secondary parkinsonism - dopamine receptor agonists and levodopa drugs, with mental disorders - antidepressants, hypnotics and sedatives. Patients with spinocerebellar ataxia are prescribed nootropics, vitamins, and metabolic stimulants.

Children with minor chorea often have psycho-emotional disorders, so they need to work with a child psychologist or psychotherapist, take anti-anxiety medication to reduce anxiety and improve sleep. In the acute period, the patient should be provided with bed rest, rest with the exclusion of sound and light stimuli.

In the chronic progressive course of chorea against the background of genetically determined pathologies, special complexes of physiotherapy exercises play an important role. Massage and electrical stimulation may be indicated. With neuroacanthocytosis, devices are needed to prevent biting of the mucous membranes of the oral cavity, treatment of wounds with antiseptics. In the final stages of hereditary diseases, constant care is required, the prevention of complications caused by immobility of patients.