Guidelines for treatment of immune-mediated cerebellar ataxias

Hiroshi Mitoma,Marios Hadjivassiliou,Jérôme Honnorat

doi:10.1186/s40673-015-0034-y

Abstract

Immune-mediated cerebellar ataxias include gluten ataxia, paraneoplastic cerebellar degeneration, GAD antibody associated cerebellar ataxia, and Hashimoto’s encephalopathy. Despite the identification of an increasing number of immune-mediated cerebellar ataxias, there is no proposed standardized therapy. We evaluated the efficacies of immunotherapies in reported cases using a common scale of daily activity. The analysis highlighted the importance of removal of autoimmune triggering factors (e.g., gluten or cancer) and the need for immunotherapy evaluation (e.g., corticosteroids, intravenous immunoglobulin, immunosuppressants) and adaptation according to each subtype.Electronic supplementary materialThe online version of this article (doi:10.1186/s40673-015-0034-y) contains supplementary material, which is available to authorized users.

Highlights

Accumulating evidence suggests that the cerebellum is one of the main CNS targets of autoimmunity, as demonstrated by the high prevalence of paraneoplastic cerebellar degeneration (PCD) amongst paraneoplastic neurological syndromes [1, 2]
Two patients with latency to treatment of 10 years responded well to corticosteroids (Patients # 2 and 9 showed full and good recovery, respectively) and both had no cerebellar atrophy on MRI. These results suggest that immune-mediated cell loss would be slow characteristically in some patients with steroid-response immune-mediated CAs (IMCAs) associated with anti-thyroid Abs, resulting in a chance of satisfactory improvement using immunomodulators
In this review, we evaluated the efficacy of each immunotherapy based on case reports and retrospective studies

Summary

Introduction

Accumulating evidence suggests that the cerebellum is one of the main CNS targets of autoimmunity, as demonstrated by the high prevalence of paraneoplastic cerebellar degeneration (PCD) amongst paraneoplastic neurological syndromes [1, 2]. In addition to the well-established concept of PCD, the clinical entity of non-paraneoplastic immune-mediated cerebellar ataxias (CAs) was established recently [5,6,7]. Other clinical entities will probably emerge in the future because some autoantibodies have been described recently in patients with cerebellar ataxia, but as only few patients have been described in each group, further works will be necessary to confirm autoimmune mechanisms in these patients (Table 1).

Objectives

Findings

Discussion

Conclusion