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.
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.
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:
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:
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:
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.
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.