Houtington's chorea: a description of the disease

Huntington Huntington is a chronic hereditary progressive neurodegenerative disease that is accompanied by mental and motor disorders. The main cause of the development of pathology is the defective gene, which contributes to the appearance in the nerve cells of a pathological protein that disrupts normal intracellular metabolism and leads to the occurrence of biochemical abnormalities in the brain. Over time, the ailment progresses, and its symptoms become heavier.

When the disease in the brain cells accumulates an increased amount of gamma-aminobutyric acid, and in the cells of the nerves of the skull increases the volume of iron and disrupts the exchange of dopamine.

Huntington's syndrome is transmitted from a sick parent who is a carrier of a substandard gene, according to the autosomal dominant type. In case of illness of one of the parents of the chorea, the risk of manifestation of the ailment in the child is fifty percent. The first symptoms of pathology appear at the age of 30-40 years.

The disease occurs in 3-7 cases per one hundred thousand people among Europeans, among other people one in a million is sick.

Huntington's chorea: symptoms

At the very beginning of the disease there are characteristic for chorea hyperkinesis, irregular, fast, erratic movements. If in the first stages, patients manage to suppress these manifestations with willpower, then as the disease develops, this ability weakens. Congenital and psychiatric disorders usually begin to notice after a certain time after the onset of the disease.

When a person has a disease, the intellect decreases and dementia develops.

In the process of progression of the disease, patients experience great difficulties if they wish to perform any purposeful movements. For example, when walking, a person performs unnecessary gestures, performs squats, grimaces, staggers while moving. In patients, speech becomes difficult, muscle tone decreases, and dementia gradually develops. A person can not hold a busy posture for a long time (clenching his fist, fixing his eyes on the object).

Endocrine and neurotrophic disorders may occur.

Chorea Huntington: Diagnosis

Diagnosis is based on clinical symptoms and family history. In the process of research, diffuse and atrophic changes in the brain of the head are determined. When diagnosed, chorea is separated from Alzheimer's, Parkinson's, syphilis, encephalitis, brain tumors that have similar symptoms.

Chorea Huntington: treatment

Today, there are no methods of treating the disease that would have allowed to eliminate all manifestations of Huntington's syndrome or stopped its progression. In most cases, symptomatic therapy is performed. To suppress hyperkinesis, patients take special medicines.

When depression occurs, antidepressants and psychotherapy are prescribed. To ease the condition of patients prescribe antipsychotic (antipsychotics) drugs, tranquilizers, sympathomimetics, antidepressants.

The disease is complicated by infectious manifestations, aspiration pneumonia may occur .

The average life expectancy of people suffering from the disease is 15 years after the discovery of the first signs of ailment. Usually, with the development of primary symptoms at an earlier age, the pathology progresses faster. So, if for the first time signs of the disease are noticed in patients under 20 years, their life expectancy does not exceed 8 years. Most often, death comes from concomitant complications. A fifth of patients commit suicide.