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Genetic Choreas

  • Chapter
Movement Disorder Genetics

Abstract

The “genetic choreas” consist of a heterogeneous group of disorders, similar in that chorea is typically the most prominent or presenting clinical feature and that degeneration of the striatum is the most characteristic neuropathological feature. Here we discuss the prototypical hereditary choreiform disorder, Huntington’s disease (HD), and a series of other familial disorders in which chorea is more or less part of the clinical syndrome and that may resemble HD, including Huntington’s disease-like 2 (HDL2), the neuroacanthocytoses, benign hereditary chorea, the iron accumulation diseases, and paroxysmal movement disorders. We conclude by providing comprehensive tables of the dominant and nondominant genetic choreas.

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Authors and Affiliations

  1. Department of Neurology, James J. Peters Veterans Affairs Medical Center, Bronx, NY, 10468, USA

    Ruth H. Walker MB, ChB, PhD

  2. Department of Psychiatry, Johns Hopkins University, Baltimore, MD, 21210, USA

    Dobrila D. Rudnicki PhD

  3. Department of Psychiatry, Johns Hopkins University School of Medicine, Baltimore, MD, 21287, USA

    Russell L. Margolis MD

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  1. Ruth H. Walker MB, ChB, PhD
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  2. Dobrila D. Rudnicki PhD
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  3. Russell L. Margolis MD
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Correspondence to Russell L. Margolis MD .

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Editors and Affiliations

  1. Department of Neurology, University Hospital Kiel, Kiel, Germany

    Susanne A. Schneider

  2. Department of Molecular Neuroscience, University College London, Institute of Neurology, London, United Kingdom

    José M. Tomás Brás

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Walker, R.H., Rudnicki, D.D., Margolis, R.L. (2015). Genetic Choreas. In: Schneider, S., Brás, J. (eds) Movement Disorder Genetics. Springer, Cham. https://doi.org/10.1007/978-3-319-17223-1_8

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