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Current Neuropharmacology

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ISSN (Print): 1570-159X
ISSN (Online): 1875-6190

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Review Article

Immune-mediated Cerebellar Ataxias: Practical Guidelines and Therapeutic Challenges

Author(s): Hiroshi Mitoma*, Mario Manto and Christiane S. Hampe

Volume 17, Issue 1, 2019

Page: [33 - 58] Pages: 26

DOI: 10.2174/1570159X16666180917105033

Price: $65

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.

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