Immune-mediated ataxias: Guide to clinicians

Immune-mediated cerebellar ataxias were initially described as a clinical entity in the 1980s, and since then, an expanding body of evidence has contributed to our understanding of this topic. These ataxias encompass various etiologies, including postinfectious cerebellar ataxia, gluten ataxia, paraneoplastic cerebellar degeneration, opsoclonus-myoclonus-ataxia syndrome and primary autoimmune cerebellar ataxia. The increased permeability of the brain-blood barrier could potentially explain the vulnerability of the cerebellum to autoimmune processes. In this manuscript, our objective is to provide a comprehensive review of the most prevalent diseases within this group, emphasizing clinical indicators, pathogenesis, and current treatment approaches. •Immune-mediated processes can give rise to disabling cerebellar ataxia.•The immune-mediated cerebellar ataxias are potentially treatable.•The early treatment in autoimmune cerebellar conditions is imperative.•Acute cerebellar ataxia with vermian involvement should raise suspicion.