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Neuroimmune Diseases pp 599–620Cite as

Autoimmune Ataxias

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Part of the book series: Contemporary Clinical Neuroscience ((CCNE))

Abstract

The dramatic progress to genetically characterise the ataxias, using next-generation sequencing (NGS), has also facilitated our appreciation that a substantial number of sporadic ataxias are not due to genetic defects but likely to be immune-mediated. At the same time, the recent identification of an increasing number of antibodies linked to sporadic ataxias has aided the diagnostic pathway for immune-mediated cerebellar ataxias (IMCAs). However, the diagnosis of IMCA remains problematic if it is solely dependent on the serological screening for such antibodies and also because there is significant phenotypic overlap with non-immune forms of ataxia. In the majority of cases, serological screening for known antibodies associated with IMCA may not be readily available. In others no specific antigenic trigger or associated antibodies have been identified as yet. Therefore, recognition of IMCA relies on clinical expertise, indirect evidence of autoimmunity (additional autoimmune diseases or family history of autoimmune disease) and appropriate investigations. It is imperative to consolidate quickly such a diagnosis as therapeutic interventions can be effective in preserving the cerebellar reserve.

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Author information

Authors and Affiliations

  1. Academic Department of Neurosciences, Royal Hallamshire Hospital, Sheffield, UK

    Marios Hadjivassiliou

  2. Medical Education Promotion Center, Tokyo Medical University, Tokyo, Japan

    Hiroshi Mitoma

  3. Department of Neurology, CHU-Charleroi, Charleroi, Belgium

    Mario Manto

  4. Department of Neurosciences, University of Mons, Mons, Belgium

    Mario Manto

Authors
  1. Marios Hadjivassiliou
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  2. Hiroshi Mitoma
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  3. Mario Manto
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Corresponding author

Correspondence to Marios Hadjivassiliou .

Editor information

Editors and Affiliations

  1. Medical Education Promotion Center, Tokyo Medical University, Tokyo, Japan

    Hiroshi Mitoma

  2. Department of Neurology, CHU-Charleroi, Charleroi, Belgium, Department of Neurosciences, University of Mons, Mons, Belgium

    Mario Manto

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Hadjivassiliou, M., Mitoma, H., Manto, M. (2019). Autoimmune Ataxias. In: Mitoma, H., Manto, M. (eds) Neuroimmune Diseases. Contemporary Clinical Neuroscience. Springer, Cham. https://doi.org/10.1007/978-3-030-19515-1_19

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