Huntington disease

G6_HUNTINGTON

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)']

Endpoint definition

FinnGen phenotype data

392423 individuals

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Apply sex-specific rule None

392423

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Check conditions None

392423

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Check pre-conditions, main-only, mode, registry filters

Hospital Discharge: ICD-10 G10
Hospital discharge: ICD-9 3334A
Hospital discharge: ICD-8 33100
Cause of death: ICD-10 G10
Cause of death: ICD-9 3334A
Cause of death: ICD-8 33100

2 out of 7 registries used, show all original rules.

35

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Check minimum number of events None

35

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Include endpoints None

35

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Remove individuals based on genotype QC

33

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G6_HUNTINGTON

Control definitions

Control exclude G6_NEUATR

Extra metadata

Level in the ICD hierarchy 3
First used in FinnGen datafreeze DF2
Parent code in ICD-10 G10-G13
Name in latin Morbus Huntington

Summary Statistics

Key figures

All Female Male
Number of individuals 33 18 15
Unadjusted prevalence (%) 0.01 0.01 0.01
Mean age at first event (years) 52.61 54.61 50.21

Mortality

Follow-up Absolute risk HR [95% CI] p N
1998–2019 - - - -
15 years - - - -
5 years - - - -
1 year - - - -

Age distribution of first events

Year distribution of first events

Cumulative Incidence

Correlations

Index endpoint: G6_HUNTINGTON – Huntington disease
GWS hits:

Survival analyses between endpoints

Plot

before Huntington disease
after Huntington disease

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Drugs most likely to be purchased after Huntington disease

Endpoint not on priority list, no data to show.