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)

Also Known As:

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

Nervous System Diseases: 6158
- Neurodegenerative Diseases: 16953
  - Nervous System Heredodegenerative Disorders: 36
    - Huntington Disease: 5093
- Central Nervous System Diseases: 1649
  - Movement Disorders: 4614
    - Dyskinesias: 4955
      - Chorea: 1115
        Huntington Disease: 5093
  - Brain Diseases: 2114
    - Dementia: 18504
      - Huntington Disease: 5093
    - Basal Ganglia Diseases: 227
      - Huntington Disease: 5093
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 668
- Inborn Genetic Diseases: 842
  - Nervous System Heredodegenerative Disorders: 36
    - Huntington Disease: 5093
Mental Disorders: 2989
- Cognitive Disorders Amnestic Dementia Delirium: 53
  - Dementia: 18504
    - Huntington Disease: 5093
  - Cognition Disorders: 25
    - Huntington Disease: 5093

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