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Mitoma et al. Cerebellum Ataxias (2015) 2:14 DOI 10.1186/s40673-015-0034-yREVIEWOpen AccessGuidelines for treatment of immunemediated cerebellar ataxiasHiroshi Mitoma1*, Marios Hadjivassiliou2 and J e Honnorat3,4,5,AbstractImmune-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: Cerebellar ataxias, Gluten ataxia, Paraneoplastic cerebellar degeneration, Hashimoto's encephalopathy, Anti-GAD antibodies, ImmunotherapyBackground Accumulating evidence suggests that the cerebellum is one of the main CNS targets of autoimmunity, PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/25751659 as demonstrated by the high prevalence of paraneoplastic cerebellar degeneration (PCD) amongst paraneoplastic neurological syndromes [1, 2]. Since the first description of PCD in 1919 [3], a variety of autoantibodies have been identified associated with different neoplasms [4]. In addition to the well-established concept of PCD, the clinical entity of non-paraneoplastic immune-mediated cerebellar ataxias (CAs) was established recently [5?]. In the 1980s, some of these cases were reported in association with autoantibodies, and three clinical entities have been established since then, based on the type of the associated antibodies (Abs) and specific clinical features: gluten ataxia (GA), anti-glutamic acid decarboxylase antibodies (GAD Abs)associated cerebellar ataxia (GAD Abs-CA), and Hashimoto's encephalopathy (HE). 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). Interestingly, many of these autoantibodies recognize* Correspondence: mitoma@tokyo-med.ac.jp 1 Department of Medical Education, Tokyo Medical University, Tokyo, Capivasertib Japan Full list of author information is available at the end of the articlecerebellar specific antigens located in Purkinje cell soma to dendrites resulting in an immunohistochemical staining pattern of `Medusa head' and suggesting a common entity (see Table 1) [8?0]. Prospective studies by Hadjivassiliou et al. [11] showed a prevalence of immune-mediated CAs of 32 among 320 patients with sporadic ataxia (GA 27 , PCD 3 , and GAD Abs-CA 3 ). This suggests a higher than expected incidence of immune-mediated CAs amongst sporadic CAs. Thus, clinicians are currently required to establish the diagnosis of immune-mediated CAs (IMCAs) and to initiate immunotherapy at an early stage [1, 10]. However, there is still uncertainty regarding the type of immunotherapy that should be used for each subtype of immune-mediated CAs. This can be explained by the following two reasons. Firstly, there are no large-scale randomized studies.