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 ?
12) A doyen of South African neurology commented in 1973 that he had not encountered Huntington's chorea in black patients.
Screening is more controversial for other rare genetic diseases such as Huntington's chorea, a form of physical and mental deterioration.
For example, some scientists speculate that the natural marijuana-like substance may play a role in Huntington's chorea, a progressive hereditary disease that interferes with muscular control.
Huntington's chorea - clinical presentation and treatment
They would screen out perfectly healthy men who had first-degree relatives with hemophilia--apparently not recognizing that if they were healthy they couldn't have the hemophilia mutation--but would allow in men who had parents with Huntington's chorea and who were young enough not to have presented [with symptoms].
In addition, Wolf Reik of Cambridgesays that, because methylation has been implicated in regulating the expression of genes, the relative number of methyl groups may affect the outcome of a particular gene, such as the gene for Huntington's chorea.
is approved for the treatment of movement disorders associated with Huntington's chorea and hemiballismus, and EUSA Pharma has commercial rights to the product in France.
Current programs of the company address such conditions as Parkinson's disease, epilepsy and Huntington's chorea, all of which are large markets not adequately served by current therapeutic options.
In Canada, Nitoman has been available since 1996 and is indicated for a number of hyperkinetic movement disorders, including Huntington's chorea, Tourette Syndrome and tardive dyskinesia.
Current programs of the company address such conditions as Parkinson's disease, Huntington's chorea and epilepsy, all of which are large markets not adequately served by current therapeutic options.
NBI-98854 may also be useful in other disorders such as Huntington's chorea, schizophrenia, Tourette's syndrome, and tardive dystonia.
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