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Immune-mediated Cerebellar Ataxias: Practical Guidelines and Therapeutic Challenges

[ Vol. 17 , Issue. 1 ]

Author(s):

Hiroshi Mitoma*, Mario Manto and Christiane S. Hampe   Pages 33 - 58 ( 26 )

Abstract:


Immune-mediated cerebellar ataxias (IMCAs), a clinical entity reported for the first time in the 1980s, include gluten ataxia (GA), paraneoplastic cerebellar degenerations (PCDs), antiglutamate decarboxylase 65 (GAD) antibody-associated cerebellar ataxia, post-infectious cerebellitis, and opsoclonus myoclonus syndrome (OMS). These IMCAs share common features with regard to therapeutic approaches. When certain factors trigger immune processes, elimination of the antigen( s) becomes a priority: e.g., gluten-free diet in GA and surgical excision of the primary tumor in PCDs. Furthermore, various immunotherapeutic modalities (e.g., steroids, immunoglobulins, plasmapheresis, immunosuppressants, rituximab) should be considered alone or in combination to prevent the progression of the IMCAs. There is no evidence of significant differences in terms of response and prognosis among the various types of immunotherapies. Treatment introduced at an early stage, when CAs or cerebellar atrophy is mild, is associated with better prognosis. Preservation of the “cerebellar reserve” is necessary for the improvement of CAs and resilience of the cerebellar networks. In this regard, we emphasize the therapeutic principle of “Time is Cerebellum” in IMCAs.

Keywords:

Cerebellar ataxias, immune-mediated cerebellar ataxias, prognosis, therapy, treatment, immunotherapy, gluten ataxia, paraneoplastic cerebellar degeneration, anti-GAD65Ab-associated cerebellar ataxia, post-infectious cerebellitis, opsoclonus myoclonus syndrome.

Affiliation:

Medical Education Promotion Center, Tokyo Medical University, Tokyo, Service des Neurosciences, UMons, 7000 Mons, University of Washington, School of Medicine, Seattle, WA 98109

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