10. Huntington Disease and Other Genetic Choreas

  1. Alberto Albanese MD1 and
  2. Joseph Jankovic MD2
  1. Ainhi Ha and
  2. Joseph Jankovic MD

Published Online: 24 FEB 2012

DOI: 10.1002/9781444346183.ch10

Hyperkinetic Movement Disorders: Differential Diagnosis and Treatment

Hyperkinetic Movement Disorders: Differential Diagnosis and Treatment

How to Cite

Ha, A. and Jankovic, J. (2011) Huntington Disease and Other Genetic Choreas, in Hyperkinetic Movement Disorders: Differential Diagnosis and Treatment (eds A. Albanese and J. Jankovic), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444346183.ch10

Editor Information

  1. 1

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

  2. 2

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

Author Information

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

Publication History

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

ISBN Information

Print ISBN: 9781444333527

Online ISBN: 9781444346183

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Keywords:

  • HD, and other genetic choreas;
  • HD, autosomal, neurodegeneration;
  • premanifest HD;
  • psychiatric, in premanifest;
  • motor disorder, genetic testing;
  • Westphal variant;
  • adult-onset HD;
  • HD and neuropathology

Summary

Huntington disease (HD) is an autosomal dominant, neurodegenerative disease that results from an expanded and unstable trinucleotide repeat in the IT15 gene on the short arm of chromosome 4. Clinical manifestations include disorders of motor control, classically chorea, although other movement disorders may also occur. Cognitive and behavioral disturbances are common and may contribute to significant functional decline. In the setting of a compatible clinical picture and a negative gene test, HD phenocopies should be considered, although these account for only a small proportion of cases.