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
References in periodicals archive ?
Factors influencing age at onset and duration of survival in Huntington's chorea. Ann Hum Genet 1981;45:387-96.
Guthrie's career was cut short by Huntington's chorea, a hereditary degenerative disease, which caused him to die a slow, lingering death.
Among singers and song writers he associated with were Huddie Ledbetter and Pete Seeger, and, as he lived out his last years in a hospital, dying of Huntington's Chorea, and younger singers who began to pick up and extend his voice and manner, including Jack Elliott and <IR> BOB DYLAN </IR> .
In Huntington's chorea, the lay societies play an extremely important role.
As we roll into 1964, the nurses are faced with leprosy, stroke, Huntington's chorea, cataracts and unmarried mothers.
The origin of Huntington's chorea in the Afrikaner population of South Africa.
The former Roath clinic district nurse also admitted visiting the pensioner, aged in her 80s, who was named only as Mrs A, during her lunch hours in her University Trust uniform - feeling sorry that she was seeing a second male relative die of Huntington's Chorea and taking her daughter, then aged five, on social visits.
25 of complications from Huntington's chorea. She was 76.
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).
All 12 members of the Food and Drug Administration's Peripheral and Central Nervous System Drugs Advisory Committee agreed that the available data supported the approval of tetrabenazine for reducing Huntington's chorea. In a small study comparing the drug with placebo, the drug had a robust effect on chorea, the primary outcome, but did not improve secondary outcomes measuring functionality and cognition.
He had already been struggling to come to terms with the life-threatening genetic disorder Huntington's chorea. He lost his father Arthur, 63, and brothers Brian, 59, and David, 57, to the disease.

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