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Klinische Neurologie pp 1–12Cite as

Degenerative Ataxien

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Zusammenfassung

Die degenerativen Ataxien umfassen ein weites Spektrum von z. T. erblichen neurodegenerativen Erkrankungen mit unterschiedlichen klinischen Charakteristika und heterogener Neuropathologie. Die erblichen Formen werden nach dem Erbgang in autosomal-rezessive Ataxien, deren Hauptvertreter die Friedreich-Ataxie ist, und in autosomal-dominante Ataxien (SCA, EA) unterteilt. Aufgrund aktueller Forschungsergebnisse ist bei einem Teil dieser Erkrankungen heute eine molekulargenetische Diagnosesicherung möglich. Von den nichterblichen degenerativen Ataxien werden die idiopathischen, sporadischen Fälle (SAOA, MSA-C) besprochen. Der Verlauf bei den hereditären und sporadischen degenerativen Ataxien ist in der Regel chronisch progredient, eine wirksame kausale Therapie gibt es bisher nicht. Um eine kausal therapierbare Ursache nicht zu übersehen, sollte eine Vitamin-E, -B12-, Schilddrüsenhormon- AFP, Laktat, VLCFA, Lysosomale Enzyme, GAD-AK, antineuronale AK und Anti-Gliadin-AK-Bestimmung sowie eine Lumbalpunktion erfolgen.

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Authors and Affiliations

  1. Neurologische Klinik, Städtisches Klinikum Braunschweig gGmbH, Braunschweig, Deutschland

    Karl Wessel & Tobias Weiland

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  1. Karl Wessel
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  2. Tobias Weiland
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Correspondence to Karl Wessel .

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Editors and Affiliations

  1. Essen, Germany

    Peter Berlit

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Wessel, K., Weiland, T. (2019). Degenerative Ataxien. In: Berlit, P. (eds) Klinische Neurologie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-44768-0_138-2

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  • DOI: https://doi.org/10.1007/978-3-662-44768-0_138-2

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-44768-0

  • Online ISBN: 978-3-662-44768-0

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Chapter history

  1. Latest

    Degenerative Ataxien
    Published:
    21 January 2019

    DOI: https://doi.org/10.1007/978-3-662-44768-0_138-2

  2. Original

    Degenerative Ataxien
    Published:
    11 May 2018

    DOI: https://doi.org/10.1007/978-3-662-44768-0_138-1

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