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Concept information

Preferred term

Huntington Disease  

Type

  • mesh:Descriptor

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)

Entry terms

  • Chorea, Chronic Progressive Hereditary (Huntington)
  • Chronic Progressive Hereditary Chorea (Huntington)
  • Huntington Chorea
  • Huntington Chronic Progressive Hereditary Chorea
  • Huntington's Chorea
  • Huntington's Disease
  • Progressive Chorea, Chronic Hereditary (Huntington)
  • Progressive Chorea, Hereditary, Chronic (Huntington)

In other languages

  • French

  • Chorée chronique progressive de Huntington
  • Chorée chronique progressive héréditaire de Huntington
  • Chorée de Huntington

URI

http://data.loterre.fr/ark:/67375/JVR-FXX73H4Q-B

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