Huntington disease

Description

obsolete_Huntington's 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.', '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)']

Data source
FinnGen phenocode G6_HUNTINGTON
Hospital Discharge registry ICD-10: G10, ICD-9: 3334A, ICD-8: 33100
Cause of Death registry ICD-10: G10, ICD-9: 3334A, ICD-8: 33100
Level in the ICD-hierarchy 3
First defined in version DF2
Ontology
DOID 12858
GWAS catalog 0000533

Key figures

Sex All Female Male
Number of individuals 10 7 N/A
Unadjusted prevalence (%) 0.01 0.01 N/A
Mean age at first event (years) 49.88 52.19 N/A
Median number of events / individual 1 1 N/A

Clinical metrics

Sex All Female Male
Recurrence within 6 months (%) 30.0 28.57 N/A
Case fatality at 5-years (%) 0.0 0.0 N/A

Associations