Hereditary ataxia

hereditary ataxia: A hereditary disorder consisting of degeneration of the cerebellum and/or of the spine; most cases feature both to some extent, and therefore present with overlapping cerebellar and sensory ataxia, even though one is often more evident than the other. Hereditary disorders causing ataxia include autosomal dominant ones such as spinocerebellar ataxia, episodic ataxia, and dentatorubropallidoluysian atrophy, as well as autosomal recessive disorders such as Friedreich's ataxia (sensory and cerebellar, with the former predominating) and Niemann Pick disease, ataxia-telangiectasia (sensory and cerebellar, with the latter predominating), and abetalipoproteinaemia. An example of X-linked ataxic condition is the rare fragile X-associated tremor/ataxia syndrome or FXTAS.

Endpoint definition

Name G6_HERATAXIA
Long name Hereditary ataxia
Hospital Discharge registry ICD-10: G11, ICD-9: 334, ICD-8: 332
Cause of Death registry ICD-10: G11, ICD-9: 334, ICD-8: 332
Level in the ICD-hierarchy 3
First defined in version DF2
Latin name Ataxia hereditaria

Summary Statistics

Key figures

All Female Male
Number of individuals 154 70 84
Unadjusted prevalence (%) 0.07 0.06 0.09
Mean age at first event (years) 50.41 50.13 50.65
Case fatality at 5-years (%) 4.55 4.29 4.76

Longitudinal metrics

All Female Male
Median nb. of events per indiv. 2.0 2.0 2.0
Recurrence at 6 months (%) 45.45 50.00 41.67

Survival analyses between endpoints

Plot

before Hereditary ataxia
after Hereditary ataxia

Loading survival analyses plot…

Drugs most likely to be purchased after Hereditary ataxia