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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.

PubMed Health Glossary
(Source: NIH - National Human Genome Research Institute)

About Huntington Disease

Huntington's disease (HD) is an inherited disorder that causes degeneration of brain cells, called neurons, in motor control regions of the brain, as well as other areas. Symptoms of the disease, which gets progressively worse, include uncontrolled movements (called chorea), abnormal body postures, and changes in behavior, emotion, judgment, and cognition. People with HD also develop impaired coordination, slurred speech, and difficulty feeding and swallowing.

HD typically begins between ages 30 and 50. An earlier onset form, called juvenile HD, occurs under age 20. Symptoms of juvenile HD differ somewhat from adult onset HD and include unsteadiness, rigidity, difficulty at school, and seizures.

More than 30,000 Americans have HD.

Huntington's disease is caused by a mutation in the gene for a protein called huntingtin....Read more about Huntington Disease
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.

[Cholinesterase inhibitors for rarer dementia associated with neurological conditions]

There are various rarer dementias including Huntington's disease (HD), cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), frontotemporal dementia (FTD), dementia in multiple sclerosis (MS) and progressive supranuclear palsy (PSP). A group of chemicals known as cholinesterase inhibitors are considered to be the first‐line medicines for Alzheimer's disease and some other dementias. Cholinesterase inhibitors may also lead to clinical improvement for rarer dementias associated with neurological conditions.

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Terms to know

Genes
A unit of genetic material (DNA) inherited from a parent that controls specific characteristics. Genes carry coded directions a cell uses to make specific proteins that perform specific functions.
Genetic
Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
Genetic Testing
Analyzing DNA to look for a genetic alteration that may indicate an increased risk for developing a specific disease or disorder.
Neurologist
A doctor who specializes in the diagnosis and treatment of disorders of the nervous system.

More about Huntington Disease

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

Other terms to know: See all 4
Genes, Genetic, Genetic Testing

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