Huntington's chorea


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Related to Huntington's chorea: multiple sclerosis, Parkinson's disease

Huntington's chorea (disease)

 [hunt´ing-tunz]
a rare hereditary disease characterized by quick involuntary movements, speech disturbances, and mental deterioration due to degenerative changes in the cerebral cortex and basal ganglia; it appears in adulthood, usually between the ages of 30 and 45, and the patient's condition deteriorates over a period of about 15 years to total incapacitation and death. There is not currently any treatment that can cure this disorder, although sedatives and antianxiety agents may relieve symptoms in the early stages. As the disease progresses, admission to a psychiatric facility is usually necessary. Called also chronic or hereditary chorea.

Huntington's chorea

an incurable degeneration of the human nervous system characterized by involuntary movements of the head, face and/or limbs, leading to eventual death. The disorder is caused by a single autosomal dominant gene on chromosome 4 (see DOMINANCE that shows delayed penetrance, fewer than 5% of cases being seen before the age of 25. Thus many individuals carrying the dominant allele have reproduced before their own gene shows itself; their progeny in turn may have to wait until middle age to discover if they have inherited the condition. Named after the American neurologist George Huntington (1851–1916).

Huntington's chorea

A hereditary disease that typically appears in midlife, marked by gradual loss of brain function and voluntary movement. Some of its symptoms resemble those of schizophrenia.
Mentioned in: Schizophrenia

disease

pathogenic entity characterized by an identifiable aetiological agent, group of signs and symptoms and/or consistent anatomical alterations; see syndrome
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

chorea

irregular, spasmodic, involuntary movements of limbs or facial muscles
  • Huntington's chorea; Huntington's disease see disease, Huntington's

    Sydenham's chorea; St Vitus' dance acute neurological disorder of young people triggered by group A haemolytic streptococcus infection, causing formation of autoantibodies directed against the basal ganglia; characterized by involuntary, irregular, jerky movements (of facial, neck and limb muscles) which are increased by effort and disappear during sleep

Huntington's chorea (hun´ting-tənz),

n.pr a rare, abnormal hereditary condition characterized by chronic, progressive chorea and mental deterioration that terminates in dementia. The individual afflicted usually shows the first signs in the fourth decade of life and dies usually within 15 years. There is no known effective treatment but symptoms can be relieved with medications.
References in periodicals archive ?
Factors influencing age at onset and duration of survival in Huntington's chorea.
Many people who many into families with Huntington's chorea are not informed about the threat to the health of spouse and children.
I hear they've lined up a spot of leprosy, strokes, Huntington's chorea & cataracts
The most common adverse events reported during the 12-month period were falls (14% of patients), Huntington's chorea, originally reported as a worsening of pre-existing chorea (13% of patients), depression (8% of patients), dizziness, nasopharyngitis, fatigue, irritability (each reported for 7% of patients), diarrhoea, nausea and insomnia (each reported for 6% of patients).
Tetrabenazine was first approved in 1971 in the United Kingdom for treating organic movement disorders and tardive dyskinesia, and was approved for treating Huntington's chorea in France in 2005 and in the Netherlands and Germany in 2007.
He had already been struggling to come to terms with the life-threatening genetic disorder Huntington's chorea.
The company is working with seven of New Zealand's research institutes to develop a new substance, mitoquinone, for treating Friedreich's Ataxia and Huntington's Chorea.
In another case, a man was jailed for three years for raping a terminally- ill woman suffering from the degenerative brain disorder Huntington's chorea, at a nursing home.
The case involves a grandfather who has Huntington's chorea.
For example, the majority of patients who are at risk for Huntington's chorea do not get tested because they don't want a death sentence, and there's nothing they can do about it.
Sometimes residents are placed inappropriately; I remember one facility that had a Huntington's chorea case it didn't know how to handle, and was cited for immediate jeopardy.

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