Last reviewed 01/2018

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