Epidemiology

Epidemiological studies of the progressive ataxias in the UK are sparse

• estimated prevalence: 1 in 12,500 adults with autosomal dominant cerebellar ataxia in the North East of England
• estimated minimum prevalence: 10.2 in 100,000 people with late onset cerebellar ataxia in South Wales
• most common inherited ataxia in the UK is Friedreich’s ataxia, which is a recessively inherited condition that tends to be of early onset
  • estimated prevalence of Friedreich’s ataxia in studies before the availability of genetic tests: 1 in 50,000
    • a more recent study estimated a carrier frequency of 1 in 85 and a disease incidence of 1 in 29,000
• studies suggest that the prevalence of the progressive ataxias is higher than conditions that are generally better known such as Huntington’s disease and motor neurone disease

Reference:

• (1) Ataxia UK (March 2007). Management of Ataxia: Guidelines on Best Clinical Practice.