Guidelines for treatment of immune-mediated cerebellar ataxias

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.

Keywords: Anti-GAD antibodies; Cerebellar ataxias; Gluten ataxia; Hashimoto’s encephalopathy; Immunotherapy; Paraneoplastic cerebellar degeneration.

Fig. 1

The clinical time-course could be classified into 6 patterns; a full recovery type, b partial recovery type, c stabilization type, d fluctuated type, e gradually progressive type, and f rapidly progressive type. Triangle indicates the time of immunotherapy. Possible clinical courses for each subtype are schematically indicated below. Black indicates ‘common course’, gray ‘occasional course’, and light gray ‘rare course’. GA gluten ataxia, PCD paraneoplastic cerebellar degeneration, GAD anti-GAD Antibodies associated cerebellar ataxia. HE Hashimoto’s encephalopathy, The clinical entity of HE is still controversial. Thus, we show the time-course of steroid-response IMCA associated with anti-thyroid Abs.