Chorea Huntington is a chronic genetic disorder of the nervous system that can develop both in childhood and adulthood, but most often begins to manifest in people aged 30 to 50 years. This is a serious, slowly progressing disease, which is characterized by various degenerative processes in the body, affecting the brain more.
Causes of Huntington's chorea
As already noted, Huntington's chorea is a genetic disease, so it is inherited from sick parents. The type of inheritance of Huntington's chorea is autosomal dominant. Pathology is more common in men. It is also known that a certain role in the development of Huntington's chorea is played by the transmitted infections, trauma, drug intoxication.
The gene hantingtin, located in all people on the fourth chromosome, is responsible for the coding of the eponymous protein, whose functions are not known precisely for today. This protein is found in the neurons of various parts of the brain. The disease develops when the gene changes due to the lengthening of the chain of amino acids. When a certain amount of amino acids is reached, the protein begins to exert a toxic effect on the cells of the body.
Symptoms of Huntington's chorea
The disease is characterized by gradually growing symptoms, which include:
- intellectual disorders - decreased memory, attention, ability to think, personality change;
- physical disorders - choreic hyperkinesis, characterized by fast, erratic and irregular movements of different muscle groups (mainly, aimless twitching of the muscles of the face, arms and shoulders, spasmodic movements of the eyes, changes in gait);
- difficult, slurred speech;
- sleep disorders ;
- difficulty of voluntary movements;
- reducing criticism to one's own behavior and condition;
- unreasonable attacks of panic, apathy and depression ;
- increased bad habits;
- hypersexuality;
- development of dementia (dementia);
- endocrine and neurotrophic disorders.
Between the appearance of neurological and psychopathological symptoms there may be a gap of several years. Over time, various complications develop: heart failure, pneumonia, cachexia. The life expectancy of patients with Huntington's chorea is different, but on average is about 15 years. The most common death is due to complications.
Treatment of Huntington's chorea
At the moment the disease is considered incurable. Medicine can only slow its progression, and also minimize the manifestation of symptoms that reduce the quality of life. To this end, patients are assigned a number of medications, including:
- Tetrabenazine - a specially developed tool for reducing the severity of symptoms of Huntington's chorea;
- antiparkinsonian means - to facilitate hypokinesia and rigidity of muscles;
- valproic acid - to relieve myoclonic hyperkinesia;
- selective serotonin reuptake inhibitors and mirtazapine - to eliminate severe depression;
- atypical antipsychotics - with psychoses and behavioral disorders;
- neuroleptics and benzodiazepines - to reduce the manifestation of chorea;
- antagonists of dopamine - to suppress hyperkinesis;
- preparations valproic acid - with myoclonic cramps.
Some of the above drugs are banned for use in our country, despite their high efficiency. Therefore, many patients turn to specialized clinics abroad for treatment.