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Chapter 12 - Autoimmune Cerebellar Ataxias

from Section 3 - Specific Syndromes and Diseases

Published online by Cambridge University Press:  27 January 2022

Josep Dalmau  and
Francesc Graus
Josep Dalmau
Affiliation:
Universitat de Barcelona
Francesc Graus
Affiliation:
Universitat de Barcelona
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Summary

Autoimmune cerebellar ataxias include a heterogeneous group of disorders characterized by isolated or predominant cerebellar dysfunction caused by immune-mediated mechanisms. The best-characterized autoimmune ataxia is paraneoplastic cerebellar degeneration (PCD) that, depending on the type of cancer, associates with different paraneoplastic antibodies such as Yo antibodies in patients with breast or ovarian cancer, Tr (DNER) antibodies in patients with Hodgkin lymphoma, and SOX1 or voltage-gated calcium channel (VGCC) antibodies in patients with small-cell lung cancer (SCLC). Patients with PCD and SCLC can have concurrent symptoms of Lambert–Eaton myasthenic syndrome (LEMS). Non-paraneoplastic cerebellar ataxias usually associate with glutamic acid decarboxylase (GAD) or mGluR1 antibodies. Patients with autoimmune ataxia respond poorly to immunotherapy even when the associated antibodies are directed against neuronal surface antigens (VGCC, mGluR1). Cerebellar ataxia may occur in patients with dietary gluten sensitivity. The autoimmune pathogenesis of gluten ataxia is unclear. Acute cerebellar ataxia and acute cerebellitis are the most frequent causes of cerebellar dysfunction in children. Whereas the term acute cerebellar ataxia is used to define patients with normal MRI and a benign clinical course, the term acute cerebellitis implies a more severe disorder with MRI inflammatory changes. In these patients the long-term prognosis is less favourable.

Keywords

Paraneoplastic cerebellar degenerationYo antibodiesTr (DNER) antibodiesSox1 antibodiesvoltage-gated calcium channel antibodiesGAD antibodiesmGluR1 antibodiesgluten ataxiaacute cerebellitis
Type
Chapter
Information
Autoimmune Encephalitis and Related Disorders of the Nervous System , pp. 342 - 367
Publisher: Cambridge University Press
Print publication year: 2022

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  • Autoimmune Cerebellar Ataxias
  • Josep Dalmau, Universitat de Barcelona, Francesc Graus, Universitat de Barcelona
  • Book: Autoimmune Encephalitis and Related Disorders of the Nervous System
  • Online publication: 27 January 2022
  • Chapter DOI: https://doi.org/10.1017/9781108696722.013
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  • Autoimmune Cerebellar Ataxias
  • Josep Dalmau, Universitat de Barcelona, Francesc Graus, Universitat de Barcelona
  • Book: Autoimmune Encephalitis and Related Disorders of the Nervous System
  • Online publication: 27 January 2022
  • Chapter DOI: https://doi.org/10.1017/9781108696722.013
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  • Autoimmune Cerebellar Ataxias
  • Josep Dalmau, Universitat de Barcelona, Francesc Graus, Universitat de Barcelona
  • Book: Autoimmune Encephalitis and Related Disorders of the Nervous System
  • Online publication: 27 January 2022
  • Chapter DOI: https://doi.org/10.1017/9781108696722.013
Available formats
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