Huntingtons Symptoms - Huntingtons Disease

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About Huntingtons Disease

Huntingtons disease results from genetically programmed degeneration of brain cells, called neurons, in certain areas of the brain. This degeneration causes uncontrolled movements, loss of intellectual faculties, and emotional disturbance. Huntingtons Disease is a familial disease, passed from parent to child through a mutation in the normal gene.

Each child of an Huntingtons Disease parent has a 50-50 chance of inheriting the Huntingtons Disease gene. If a child does not inherit the Huntingtons Disease gene, he or she will not develop the disease and cannot pass it to subsequent generations. A person who inherits the Huntingtons Disease gene will sooner or later develop the disease. Whether one child inherits the gene has no bearing on whether others will or will not inherit the gene.

Some early symptoms of Huntingtons Disease are mood swings, depression, irritability or trouble driving, learning new things, remembering a fact, or making a decision. As the disease progresses, concentration on intellectual tasks becomes increasingly difficult and the patient may have difficulty feeding himself or herself and swallowing.

The rate of disease progression and the age of onset vary from person to person. A genetic test, coupled with a complete medical history and neurological and laboratory tests, help physician's diagnose Huntingtons Disease. Presymptomic testing is available for individuals who are at risk for carrying the Huntingtons Disease gene. In 1 to 3 percent of individuals with Huntingtons Disease, no family history of Huntingtons Disease can be found.

Treatment

Physicians prescribe a number of medications to help control emotional and movement problems associated with Huntingtons Disease. Most drugs used to treat the symptoms of Huntingtons Disease have side effects such as fatigue, restlessness, or hyperexcitability. It is extremely important for people with Huntingtons Disease to maintain physical fitness as much as possible, as individuals who exercise and keep active tend to do better than those who do not.

Prognosis

At this time, there is no way to stop or reverse the course of Huntingtons Disease. Now that the Huntingtons Disease gene has been located, investigators are continuing to study the Huntingtons Disease gene with an eye toward understanding how it cause disease in the human body.

Research

Scientific investigations using electronic and other technologies enable scientists to see what the defective gene does to various structures in the brain and how it affects the body's chemistry and metabolism. Laboratory animals are being bred in the hope of duplicating the clinical features of Huntingtons Disease so that researchers can learn more about the symptoms and progression of Huntingtons Disease.

Investigators are implanting fetal tissue in rodents and nonhuman primates with the hope of understanding, restoring, or replacing functions typically lost by neuronal degeneration in individuals with Huntingtons Disease.

Related areas of investigation include excitotoxicity (overstimulation of cells by natural chemicals found in the brain), defective energy metabolism (a defect in the mitochondria), oxidative stress (normal metabolic activity in the brain that produces toxic compounds called free radicals), tropic factors (natural chemical substances found in the human body that may protect against cell death).

Reference for Huntingtons Disease Article

National Institutes of Health

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