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Huntington Disease

Huntington's disease is an inherited disease characterized by the progressive loss of brain and muscle function. Symptoms usually begin during middle age. The disease is inherited.

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(Source: NIH - National Human Genome Research Institute)

About Huntington Disease

Huntington's disease (HD) 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.

HD is a familial disease, passed from parent to child through a mutation in the normal gene. Each child of an HD parent has a 50-50 chance of inheriting the HD gene. If a child does not inherit the HD gene, he or she will not develop the disease and cannot pass it to subsequent generations.

A person who inherits the HD 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 HD 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, helps physicians diagnose HD. Presymptomatic testing is available for individuals who are at risk for carrying the HD gene. In 1 to 3 percent of individuals with HD, no family history of HD can be found. NIH - National Institute of Neurological Disorders and Stroke

What works? Research summarized

Evidence reviews

Interventions to control symptoms in Huntington's disease

Huntington's disease (HD) is an autosomal dominant neurodegenerative disease. No curative therapy is currently available. We proposed to assess the effectiveness of interventions aimed at controlling the symptoms of HD and to analyse the methodological quality of the corresponding clinical trials. 22 trials were identified. The review of these trials comprising 1254 HD patients revealed that no intervention produced a robust conclusive symptomatic effect. Nevertheless, tetrabenazine was the drug for which better data exists supporting a beneficial effect in the treatment of chorea. There were no available data for the specific treatment of other clinical relevant problems associated with HD such as depression, irritability, apathy, cognitive impairment or psychosis.

Interventions to delay progression of Huntington's disease

Huntington´s disease (HD) is an autosomal dominant neurodegenerative disease for which no cure is currently available. We proposed to assess the effectiveness of interventions aimed at modifying disease progression and evaluate the methodological quality of the corresponding clinical trials. We selected eight trials comprising a total of 1366 participants. The results show that no intervention demonstrated an effect in modifying disease progression in HD.

The pathophysiology and pharmacological treatment of Huntington disease

INTRODUCTION: Huntington disease (HD) is a progressive neurodegenerative condition characterised by motor, cognitive and behavioural dysfunction, and has an autosomal dominant mode of inheritance. As there is currently no treatment to delay progression of the disease, pharmacological intervention is aimed at symptomatic relief.

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Summaries for consumers

Interventions to control symptoms in Huntington's disease

Huntington's disease (HD) is an autosomal dominant neurodegenerative disease. No curative therapy is currently available. We proposed to assess the effectiveness of interventions aimed at controlling the symptoms of HD and to analyse the methodological quality of the corresponding clinical trials. 22 trials were identified. The review of these trials comprising 1254 HD patients revealed that no intervention produced a robust conclusive symptomatic effect. Nevertheless, tetrabenazine was the drug for which better data exists supporting a beneficial effect in the treatment of chorea. There were no available data for the specific treatment of other clinical relevant problems associated with HD such as depression, irritability, apathy, cognitive impairment or psychosis.

Interventions to delay progression of Huntington's disease

Huntington´s disease (HD) is an autosomal dominant neurodegenerative disease for which no cure is currently available. We proposed to assess the effectiveness of interventions aimed at modifying disease progression and evaluate the methodological quality of the corresponding clinical trials. We selected eight trials comprising a total of 1366 participants. The results show that no intervention demonstrated an effect in modifying disease progression in HD.

More about Huntington Disease

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Also called: Huntington's disease, Huntington's chorea, HD

Other terms to know:
Genes, Genetic

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