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Autosomal recessive ataxia, Beauce type

Disease definition

A rare disorder characterised by a slowly progressive pure cerebellar ataxia associated with dysarthria. It has been described in 53 individuals from 26 families of Canadian origin. The mode of transmission is autosomal recessive. Positional cloning has led to the identification of several gene mutations.


Classification level: Disorder
  • Synonym(s):
    • ARCA1
    • Autosomal recessive cerebellar ataxia type 1
    • SCAR8
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Adult
  • ICD-10: G11.2
  • OMIM: 610743
  • UMLS: C3683483
  • MeSH: -
  • GARD: 12234
  • MedDRA: -

Detailed information

Disease review articles

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.