Huntington's Disease

Huntington's disease (HD) is an inherited disease that causes certain nerve cells in the brain to waste away. People are born with the defective gene, but symptoms usually don't appear until middle age. Early symptoms of HD may include uncontrolled movements, clumsiness or balance problems. Later, HD can take away the ability to walk, talk or swallow. Some people stop recognizing family members. Others are aware of their environment and are able to express emotions.

If one of your parents has Huntington's disease, you have a 50-50 chance of getting it. A blood test can tell you if have the HD gene and will develop the disease. Genetic counseling can help you weigh the risks and benefits of taking the test.

There is no cure. Medicines can help manage some of the symptoms, but cannot slow down or stop the disease.

National Institute of Neurological Disorders and Stroke

Huntington's Disease on Wikipedia

'''Huntington's disease''' or '''Huntington's chorea''' is an inherited disorder characterized by abnormal body movements called Chorea (disease), and loss of memory. There also is evidence that doctors as far back as the Middle Ages knew of this devastating disease. The incidence is 5 to 8 per 100,000. It takes its name from the New York physician George Huntington who first described it precisely in 1872.

Short Summary

Huntington's disease is inherited in the autosomal dominant fashion, meaning that it is on a dominant allele, and offspring of carriers have a 50% chance of inheriting the disease. (Crossing of a heterozygote with a homozygous recessive partner.) Symptoms of the disorder include loss of cognitive ability (thinking, speaking), changes in personality, jerking movements of the face and body in general and unsteady walking. These symptoms develop into dementia and cognitive decline (not mental retardation which is an older term referring to the lack of development of mental ability rather than loss of it.) and an advanced form of jerking called Chorea (disease), the Greek word for dance. It usually takes between 10-25 years for the disease to kill someone, and it is fatal 100% of the time. The disease is onset in the 30s and 40s in most cases; however there is an early childhood form that starts at the age of 2. Victims of this form rarely reach adulthood. One interesting fact about the disease is that it contributes to a chemical imbalance that leads many victims to commit suicide. This is also believed in part to be a result of the position sufferers find themselves in. Another interesting fact about the disease is that it is unusually prevalent in the small Venezuelan fishing village of Barranquitas. Families there have a high presence of the disease and geneticists and doctors view this community as invaluable in the research of the disease.

Symptoms

The symptoms of Huntington’s ... [ Read More ]

External Resources

Kissing in the Kitchen - Inspirational self-help strategies through hands-on techniques to eradicate life challenges and disasters like Huntington's Disease. Spirituality, love, peace, and joy are taught through poetry.

Section Molecular Neurogenetics - Research on genetics of polyglutamine neurodegeneration, such as Huntington and spinocerebellar ataxia 2, as well as stroke spasticity and Parkinsons disease at Goethe University Hospital, Frankfurt, Germany.

Living Cell Technology - Manufacturer living cells, for the treatment of Huntington's disease, haemophilia and insulin-dependent diabetes. Product and investor information, of company with contacts in Adelaide, South Australia.

Center for Neuroregeneration Research - Workins on developing therapies for Parkinson's disease, Alzheimer's disease, Huntington's disease, Amyotrophic Lateral Sclerosis and related disorders

ReNeuron Ltd. - Developing somatic transplantation technology for diabetes, neuronal degeneration and therapy for brain damage including stroke, Parkinson's disease, Alzheimer's and Huntington's disease. Overview of company in Guildford, England.

Amyloid and Neurodegenerative Disorders - Huntington's and Alzheimer's Diseases. Inhibition of polyglutamine aggregation. Elucidation of structure of a-beta fibrils.

MGH Center for Neurodegenerative Disease Research - Information on parkinson, huntington, alzheimer. Full text research papers, publication list, conferences and seminars.

Caring for People with Huntington's Disease - Information about caring for people with Huntington's disease, for patients, families and professionals.

CureHD Foundation - A charity site aiming to spread awareness, information, and news about Huntington's disease.

Huntington's Disease Society of America - Research, assistance, and education.

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