Huntington's chorea is a congenital hereditary disease, accompanied by the appearance of involuntary movements, a decrease in intelligence and the development of mental disorders. This disease can develop in men and women at absolutely any age, but most often the first symptoms of Huntington's chorea appear in the age range of 35-40 years.
Symptoms of Huntington's Disease
The main clinical sign of Huntington's disease is chorea, which is manifested by disordered and uncontrolled movements. At first, these are only minor disturbances in coordination with the jerky movements of the hands or feet. These movements can be either very slow or abrupt. Gradually, they grab the whole body and sit quietly, eat or dress becomes almost impossible. Subsequently, other symptoms of Huntington's disease begin to attach to this symptom:
- problems with the process of chewing and swallowing;
- speech impairment;
- problems with sleep;
- violation of muscle tone.
At an early stage, there may be minor personality disorders and cognitive functions. For example, the patient has a violation of the functions of abstract thinking. As a result, he can not plan actions, perform them and give them an adequate assessment. Then the disorders become more severe: a person becomes aggressive, sexually disinhibited, self-centered, obsessive ideas appear and addiction (alcoholism, gambling) increases.
Diagnosis of Huntington's Disease
Diagnosis of Huntington's syndrome is carried out using various methods of psychological examination and physical examination. Among the instrumental methods, the main place is occupied by magnetic resonance imaging and computed tomography. It is with their help that you can see the place of brain damage.
Genetic testing is used from screening methods. If more than 38 trinucleotide residues of the CAG are detected in the HD gene, Huntington's disease will eventually arise in 100% of cases. In this case, the smaller the number of residues, the later in later life will manifest chorea.
Treatment of Huntington's Disease
Unfortunately, Huntington's disease is incurable. At the moment, in the fight against this disease, only symptomatic therapy is applied, which temporarily facilitates the patient's condition.
The most effective drug, weakening the symptoms of the disease, is Tetrabenazine. Also in the treatment are anti-Parkinsonian drugs:
- Bromocriptine;
- Levodopa;
- Pergolid.
To eliminate hyperkinesia and relieve muscle rigidity, valproic acid is used. Treatment for depression in this disease is carried out with Prozac, Citalopram, Zoloft and other selective serotonin reuptake inhibitors. When developing psychoses, atypical antipsychotics (Risperidone, Clozapine or Amisulpride) are used.
Life expectancy in people who suffer from Huntington's disease is significantly reduced. From the moment of appearance of the first symptoms of this pathology to death can pass only 15 years. At the same time, lethal
- suicidal attempts;
- pneumonia;
- pathology of the heart.
Because this is a genetic disease, prevention itself does not exist. But from the use of screening methods (prenatal diagnostics with DNA analysis) it is not necessary to refuse, because if at the early stages of starting symptomatic treatment, you can significantly prolong the life of the patient.