Huntington's disease


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Huntington's disease

(hŭn′tĭng-tənz)
n.
A rare inherited disease of the central nervous system characterized by progressive dementia, abnormal posture, and involuntary movements. The typical age of onset is between 30 and 50 years. Also called Huntington's chorea.

Huntington's disease

Etymology: George S. Huntington, American physician, 1851-1916
a rare abnormal hereditary condition characterized by chronic progressive chorea and mental deterioration that results in dementia. An individual afflicted with the condition usually shows the first signs in the fourth decade of life and dies within 15 years. It is transmitted as an autosomal trait and becomes progressively worse in severity as the trinucleotide repeats grow in successive generations. There is no known effective treatment, but symptoms can be relieved with medication.
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Huntington's disease: dilated lateral ventricle with caudate and lentiform atrophy

Huntington's disease

Huntington's chorea Neurology An AD degenerative disease of adult onset–ages 40-50 that leads inexorably to death Clinical Slowly progressive mood and personality changes, mental deterioration, loss of coordination, chorea, cognitive decline, chronic fatigue, apathy Treatment None. See 'Escapee. ', Trinucleotide repeat disease.

Huntington's disease

A rare, dominant, genetic brain disorder caused by a defective gene on chromosome number 4 that has complete penetrance. The disease appears most often in middle-aged adults and leads to loss of nerve cells and a buildup of the neurotransmitter dopamine. This causes involuntary twitching or jerking movements of the face and body (chorea), alternating excitement and depression, and progressive DEMENTIA. The chorea can be controlled by drugs but there is no treatment for the central problem. When the disease starts in childhood the inheritance is four times as likely to be from the father than from the mother. The mechanism of the disease is unknown but it is thought that neural toxicity results from the accumulation of amino-terminal fragments containing an expanded polyglutamine region. (George Huntington, 1850–1916, American General Practitioner)

Huntington's disease

A rare hereditary condition that causes progressive chorea (jerky muscle movements) and mental deterioration that ends in dementia. Huntington's symptoms usually appear in patients in their 40s. There is no effective treatment.
Mentioned in: Movement Disorders
Huntington's disease; Huntington's chorea untreatable autosomal-dominant, relentlessly progressive chorea with dementia (due to cerebral atrophy), manifesting in middle life, leading to death within 10-12 years of onset
References in periodicals archive ?
The objective of the study was to evaluate the effectiveness, safety and tolerability of RP103 in the treatment of Huntington's disease.
is a privately-funded, not-for-profit, biomedical research organization exclusively dedicated to rapidly discovering and developing therapies that slow the progression of Huntington's disease.
HDL2 mutations are an important cause of Huntington's disease in patients with African ancestry.
Although more research is needed to know whether minocycline would be helpful for people with Huntington's disease, the scientists noted that the drug appears to be easy to take.
It was work organized by the Foundation that led to the discovery of the genetic marker for Huntington's disease in 1983.
Huntington's disease is an aggressive, neuro-degenerative disorder which causes loss of co-ordination, psychiatric problems, dementia and death.
Huntington's disease afflicts about thousands of people- typically striking in midlife - and many carry the gene that will eventually lead to it.
With RNA interference therapy, researchers for the first time have been able to attack the fundamental cause of Huntington's Disease and reduce the protein expression from the disease gene.
People with Huntington's disease gradually lose neurons in their brains as defective protein molecules clump together inside those cells.
Tiny capsules planted in the brain might be able to fend off the disabling symptoms of Huntington's disease, a study in monkeys suggests.
Global Markets Direct's, 'Huntington's Disease - Pipeline Review, H2 2012', provides an overview of the Huntington's Disease therapeutic pipeline.
The Huntington's Disease Partnering 2010-2015 report provides understanding and access to the Huntington's disease partnering deals and agreements entered into by the worlds leading healthcare companies.

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