Huntington's disease is a rare hereditary pathology. It is detected in approximately one person out of ten thousand. Huntington disease provokes a progressive disorder in the nervous system. The largest lesion is detected in two clusters of cells located deep in the medulla. It is a shell and a caudate nucleus. These areas are involved in the regulation of voluntary actions at a subconscious level. The causes of Huntington's syndrome are not exactly clear, but scientists continue to work on this issue.
Symptoms of pathology are manifested, as a rule, at the age of thirty-five to fifty years. The main manifestations accompanying Huntington's disease include:
- movement disorders;
- A progressive decrease in mental activity up to dementia.
In addition, other symptoms associated with disorders of the nervous system may occur.
In most patients (about 90%), Huntington's disease is characterized by the presence of involuntary convulsive movements (chorea). This is often the very first symptom.
Other motor disorders include stiffness or a change in muscle tone, difficulties with voluntary movements and posture, problems with the pronunciation of words (dysarthria), difficulty swallowing (dysphagia), and inappropriate eye movements of an erratic nature.
A progressive decrease in mental activity accompanying Huntington's disease can be observed in combination with the development of mental disorders. Patients, in particular, are unable to concentrate, lose their long-term and short-term memory, the ability to comprehend and find a rational solution to problems. Some patients are characterized by irritability, a change in sexual behavior, aggression, apathy, psychosis, and depression.
If signs of pathology are identified for accurate diagnosis, a family history of confirming the presence of the syndrome among relatives is necessary. The use of an expert study of the nervous system in carriers of a pathological gene allows one to determine insignificant changes in speed, control of movements and reactions long before the moment when the disorders become pronounced.
Laboratory blood tests using DNA samples are used to identify abnormalities in certain groups. These include:
- patients - carriers of the gene;
- patients with signs of the disease;
- babies in the womb.
However, it should be noted that genetic studies can provoke quite severe stress and jeopardize the further course of pregnancy.
In the process of diagnosis, a necessary condition is the exclusion of other causes of the development of pathology. These, in particular, include taking a certain category of medications, stroke, alcohol abuse for a long period.
Today, this disease is not treatable. However, therapeutic measures are used to alleviate symptoms, reduce their intensity, as well as provide patients with psychological assistance.
Among the drugs that can reduce the severity of symptoms, phenothiazines are used. They control chorea. Treatment is selected quite carefully, in accordance with the characteristics of the patient's body in order to avoid the manifestation of side effects. Other drugs, dopamine agonists, can provide control of muscle stiffness. However, as practice shows, with this pathology they are less effective than with Parkinson's disease.
To alleviate anxiety, depression, irritability and other psycho-emotional disorders, physiotherapeutic procedures are prescribed for patients.