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Episodic Ataxias

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Paroxysmal Movement Disorders

Abstract

Episodic ataxias (EA) are rare autosomal dominant channelopathies characterized by recurrent episodes of ataxia, vertigo and incoordination. So far, eight subtypes have been described with identified gene mutations in five of them (EA1, EA2, EA5, EA6 and EA8). Clinical clues, for example precipitating triggers, duration of attacks, associated interictal signs and treatment response, can aid the distinction of the different subtypes of EA. The current chapter will give an overview of clinical symptoms, genetic background and treatment for the different subtypes of EA. It will also briefly discuss the clinico-genetic characteristics of further disorders that may present with recurring bouts of paroxysmal ataxia and may, therefore, fall within the episodic ataxia spectrum.

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Author information

Authors and Affiliations

  1. Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

    Simone Zittel

  2. Movement Disorders and Body Control, Movement Disorders and Neuromodulation Unit, Department of Neurology, Charité, University Medicine Berlin, Berlin, Germany

    Christos Ganos

Authors
  1. Simone Zittel
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  2. Christos Ganos
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Correspondence to Christos Ganos .

Editor information

Editors and Affiliations

  1. Georgia Regents University, Augusta, GA, USA

    Kapil D. Sethi

  2. Department of Medicine, Surgery and Dentistry “Scuola Medica Salernitana”, University of Salerno, Baronissi, Salerno, Italy

    Roberto Erro

  3. Department of Clinical and Movement Neurosciences, UCL, Queen Square Institute of Neurology, London, UK

    Kailash P. Bhatia

Electronic Supplementary Material

Characteristic interictal myokymia in a patient with genetically confirmed EA1 (MOV 10757 kb)

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Zittel, S., Ganos, C. (2021). Episodic Ataxias. In: Sethi, K.D., Erro, R., Bhatia, K.P. (eds) Paroxysmal Movement Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-53721-0_11

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  • DOI: https://doi.org/10.1007/978-3-030-53721-0_11

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-53720-3

  • Online ISBN: 978-3-030-53721-0

  • eBook Packages: MedicineMedicine (R0)

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