J Mov Disord.  2021 Jan;14(1):10-28. 10.14802/jmd.20040.

Immune-Mediated Cerebellar Ataxias: Clinical Diagnosis and Treatment Based on Immunological and Physiological Mechanisms

  • 1Department of Medical Education, Tokyo Medical University, Tokyo, Japan
  • 2Service de Neurologie, Médiathèque Jean Jacquy, CHU-Charleroi, Charleroi, Belgium
  • 3Service des Neurosciences, University of Mons, Mons, Belgium
  • 4Academic Department of Neurosciences, Royal Hallamshire Hospital, Sheffield, UK


Since the first description of immune-mediated cerebellar ataxias (IMCAs) by Charcot in 1868, several milestones have been reached in our understanding of this group of neurological disorders. IMCAs have diverse etiologies, such as gluten ataxia, postinfectious cerebellitis, paraneoplastic cerebellar degeneration, opsoclonus myoclonus syndrome, anti-GAD ataxia, and primary autoimmune cerebellar ataxia. The cerebellum, a vulnerable autoimmune target of the nervous system, has remarkable capacities (collectively known as the cerebellar reserve, closely linked to plasticity) to compensate and restore function following various pathological insults. Therefore, good prognosis is expected when immune-mediated therapeutic interventions are delivered during early stages when the cerebellar reserve can be preserved. However, some types of IMCAs show poor responses to immunotherapies, even if such therapies are introduced at an early stage. Thus, further research is needed to enhance our understanding of the autoimmune mechanisms underlying IMCAs, as such research could potentially lead to the development of more effective immunotherapies. We underscore the need to pursue the identification of robust biomarkers.


Anti-GAD ataxia; Gluten ataxia; Immune-mediated cerebellar ataxias; Opsoclonus myoclonus syndrome; Paraneoplastic cerebellar degeneration; Postinfectious cerebellitis
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