Mitochondrial ataxias

Hilary J Vernon, Laurence A. Bindoff

Research output: Chapter in Book/Report/Conference proceedingChapter

3 Scopus citations


Ataxia is one of the most frequent symptoms of mitochondrial disease. In most cases it occurs as part of a syndromic disorder and the combination of ataxia with other neurologic involvement such as epilepsy is common. Mitochondrial ataxias can be caused by disturbance of the cerebellum and its connections, involvement of proprioception (i.e., sensory ataxia) or a combination of both (spinocerebellar). There are no specific features that define an ataxia as mitochondrial, except perhaps the tendency for it to occur together with involvement of multiple other sites, both in the nervous system and outside. In this review we will concentrate on the mitochondrial disorders in which ataxia is a prominent and consistent feature and focus on the clinical features and genetic causes.

Original languageEnglish (US)
Title of host publicationHandbook of Clinical Neurology
PublisherElsevier B.V.
Number of pages13
StatePublished - Jan 1 2018

Publication series

NameHandbook of Clinical Neurology
ISSN (Print)0072-9752
ISSN (Electronic)2212-4152


  • cerebellum
  • electron transport chain
  • heteroplasmy
  • mitochondria
  • oxidative phosphorylation

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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