autosomal dominant cerebellar ataxia

Last reviewed 01/2018

This is a heterogeneous group of adult-onset progressive ataxias which have an autosomal dominant mode of inheritance. There is often a widespread neuronal degeneration which resembles multiple system atrophy.

There are three main clinical groups:

  • spinocerebellar ataxia type 1
  • spinocerebellar ataxia type 2
  • Machado-Joseph syndrome
  • cerebellar cortical degeneration