Title:Immune-mediated Cerebellar Ataxias: Practical Guidelines and Therapeutic Challenges
Volume: 17
Issue: 1
Author(s): Hiroshi Mitoma*, Mario Manto and Christiane S. Hampe
Affiliation:
- Medical Education Promotion Center, Tokyo Medical University, Tokyo,Japan
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.
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.
