Acadian ataxia

Last reviewed 04/2022

First described by Nikolaus Friedreich in a series of five papers published from 1863 to 1877,  Friedreich's ataxia is a multisystemic neurodegenerative disorder and the most common of the hereditary ataxia syndromes (1,2).

  • the mode of inheritance is autosomal recessive
  • is the most common form of progressive ataxia in the UK(3)

Extra-neurological involvement includes:

  • hypertrophic cardiomyopathy in most patients
  • diabetes mellitus in 10%

Onset is usually during adolescence.