18. Inherited and Sporadic Ataxias

  1. Alberto Albanese MD3 and
  2. Joseph Jankovic MD4
  1. Hélio A. G. Teive1,
  2. Renato P. Munhoz1 and
  3. Tetsuo Ashizawa2

Published Online: 24 FEB 2012

DOI: 10.1002/9781444346183.ch18

Hyperkinetic Movement Disorders: Differential Diagnosis and Treatment

Hyperkinetic Movement Disorders: Differential Diagnosis and Treatment

How to Cite

Teive, H. A. G., Munhoz, R. P. and Ashizawa, T. (2011) Inherited and Sporadic Ataxias, in Hyperkinetic Movement Disorders: Differential Diagnosis and Treatment (eds A. Albanese and J. Jankovic), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444346183.ch18

Editor Information

  1. 3

    Fondazione IRCCS Istituto Neurologico Carlo Besta, Università Cattolica del Sacro Cuore, Milan, Italy

  2. 4

    Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, TX, USA

Author Information

  1. 1

    Neurology Service, Department of Internal Medicine, Hospital de Clínicas, Federal University of Paraná, Curitiba, Brazil

  2. 2

    Department of Neurology, University of Florida, Gainesville, FL, USA

Publication History

  1. Published Online: 24 FEB 2012
  2. Published Print: 3 APR 2011

ISBN Information

Print ISBN: 9781444333527

Online ISBN: 9781444346183



  • ataxia;
  • cerebellar;
  • spinocerebellar;
  • hereditary;
  • mitochondrial;
  • sporadic;
  • Friedreich;
  • short tandem repeats;
  • episodic


Degenerative ataxias are rare diseases whereas symptomatic ataxias secondary to various diseases, such as multiple sclerosis and stroke, are relatively common. Congenital non-progressive ataxias are primarily due to perinatal brain injuries and developmental disorders. Among degenerative ataxias, advances in human and molecular genetics significantly increased our knowledge in inherited ataxias whereas little is known about sporadic degenerative ataxias. The major part of this chapter will primarily cover the inherited ataxias as the available information on inherited ataxias is greater than that of other types of primary ataxic disorders. Two most important information resources for reviews of inherited ataxias are the Online Mendelian Inheritance in Man (OMIM) and GeneReview; both are available through the National Center for Biotechnology Information (NCBI) website (http://www.ncbi.nlm.nih.gov/).