Huntington's disease

Obviously, the brain is the main target of most of these rare diseases. And when there is a degeneration, searches are often limited in the development of effective treatment ... hard to live with a disease whose signs continue to worsen.

What disease is it?

Huntington's disease is a rare disease characterized by a deterioration of certain nerve cells in the brain, causing engine problems, but also cognitive and mental. Signs usually appear around the age of 35 years to 50 years and change throughout life. Forms before 21 years (juvenile form) are extremely rare.

Huntington's disease is a rare disease in France, nearly 6,000 people are reached.

Huntington's disease is a hereditary genetic disease that is passed from parents to children due to the constant transmission of a specific defective gene per generation.

What are the signs of Huntington's disease?

Signs as well as the evolution of the disease vary greatly from one individual to another. More severe in some signs may not occur in others.

Motor disorders

Movement disorders are characterized by the existence of involuntary and irregular movements which may affect all body muscles causing problems with balance, dysarthria and phonation disorders, respiratory genes, swallowing disorders , blunders in gestures ... The jerking of muscles are called "chorea" and may be exaggerated by fatigue, stress and anxiety. Chorea is similar to muscle spasms.

Other movement disorders are also seen in some patients with dystonia (involuntary and uncontrollable movements), akinesia (inability to move), the inability to stay on the same position, the lack of coordination in the movements. These disorders may worsen for causing trouble walking.

Cognitive impairment

Cognitive problems include all the problems encountered by the patient during intellectual activities. These are disorders of memory, impaired concentration and attention, intellectual delays and difficulties in the ability to think, and later gradually leading to a form of dementia. The cognitive change very slowly, but generally begin with motor disorders.

Mental disorders

Mental and psychiatric disorders are generally characterized by abnormal behavior such as irritability, agitation or aggression. These behavioral problems usually occur in the context of depression, anxiety or mood jumps. Mental disorders do not occur consistently in a patient. They appear to be in crisis at the beginning of the disease or intermittently during the course of the disease.

Other signs

These key signs can cause other events in patients with weight loss as weight loss or sleep disorders.

The treatment ...

Currently, there are still no drugs that can cure the disease. The therapeutic management are primarily focused in improving signs and relief of patients. However, these drugs do not stop the progression of the disease.

Huntington's disease requires a multi-sectoral support by involving several specialists as

- Neuropsychiatrist, who is responsible for prescribing basic medicines to improve the main signs. Motor disturbances are essentially relieved by neuroleptics.

- The physiotherapist, and the physiotherapist involved rehabilitations during gait, impaired balance ...- Speech therapist, supports voice and articulation and speech.

Psychosocial support is required to assist and support the patient in his daily life.