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Huntington Disease
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Definition of 'Huntington Disease'

A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)

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Monday, November 23, 2009

Lycopene modulates nitric oxide pathways against 3-nitropropionic acid-induced neurotoxicity.

10 Oct 2009 AIM: The present study has been designed to investigate the involvement of the nitric oxide mechanism in the protective effect of lycopene against 3-nitropropionic acid-induced Huntington's disease-like symptoms in rats. MAIN METHODS: The present ... Read more...


Normal and mutant HTT interact to affect clinical severity and progression in Huntington disease.

21 Sep 2009 OBJECTIVE: Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by a CAG repeat expansion in the HD gene (HTT). We aimed to assess whether interaction between CAG repeat sizes in the mutant and normal allele could ... Read more...


Striatal expression of a calmodulin fragment improved motor function, weight loss, and neuropathology in the R6/2 mouse model of Huntington's disease.

14 Sep 2009 Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder, caused by a polyglutamine expansion in the huntingtin protein (htt). Increasing evidence suggests that transglutaminase (TGase) plays a critical role in the ... Read more...

 

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Technical information about 'Huntington Disease'

Definition: A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)

Descriptor UI: D006816

Alternative terms: Huntington Disease; Huntington Chorea; Chorea, Huntington; Huntington's Disease; Chronic Progressive Hereditary Chorea (Huntington); Huntington Chronic Progressive Hereditary Chorea; Progressive Chorea, Chronic Hereditary (Huntington); Progressive Chorea, Hereditary, Chronic (Huntington); Huntington's Chorea; Chorea, Huntington's; Chorea, Chronic Progressive Hereditary (Huntington); Huntington Disease, Late Onset; Late-Onset Huntington Disease; Huntington Disease, Late-Onset; Late Onset Huntington Disease; Juvenile Huntington Disease; Juvenile-Onset Huntington Disease; Juvenile Onset Huntington Disease; Huntington Disease, Juvenile-Onset; Huntington Disease, Juvenile Onset; Huntington Disease, Juvenile; Akinetic-Rigid Variant of Huntington Disease; Akinetic Rigid Variant of Huntington Disease; Huntington Disease, Akinetic-Rigid Variant; Huntington Disease, Akinetic Rigid Variant;

Allowable Qualifiers: blood; cerebrospinal fluid; chemically induced; classification; complications; diagnosis; diet therapy; drug therapy; economics; embryology; enzymology; ethnology; etiology; genetics; history; immunology; metabolism; microbiology; mortality; nursing; epidemiology; parasitology; pathology; physiopathology; prevention & control; psychology; radiography; radionuclide imaging; radiotherapy; rehabilitation; surgery; therapy; urine; veterinary; ultrasonography; virology;

Tree Number: C10.228.140.079.545; C10.228.140.380.278; C10.228.662.262.249.750; C10.574.500.497; C16.320.400.430; F03.087.250.400; F03.087.400.390;

History Note: 2000(1963)

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