Dies and (2) responsiveness to corticosteroids [1, 6, 57?2]. A series of reports have shown

Dies and (2) responsiveness to corticosteroids [1, 6, 57?2]. A series of reports have shown underlying immune-mediated mechanisms. However, its clinical entity is still controversial because of no evidence establishing a direct correlation between antithyroid antibodies (Abs) and ataxia given the high prevalence of anti-thyroid Abs in the normal population [1]. Consequently, there is overlapping between HE, diagnosed based on the above criteria, and other subtypes of IMCAs. Indeed, patients with GA or GAD Abs-CA often have anti-thyroid Abs [17, 52]. Pathophysiological features of patient’s CSF are also heterogeneous [63]. Taken together, further long-term systematic studies are needed to examine how steroid-responsive IMCA associated with anti-thyroid Abs (in the absence of anti-GAD and gluten antibodies) is clinically categorized. On the other hand, there is a group of patients with anti-thyroid Abs who showed characteristically good response to immunotherapies when compared with other subtypes of IMCAs [61]. Interestingly, PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/15501003 some patients showed full recovery. Table 3 summarizes the effects of immunotherapies (corticosteroids and intravenous immunoglobulins (IVIg)) in 13 patients reported by Matsunaga et al. [61]. Corticosteroids were administrated in 12 of these patients. Before the therapy, their CAs severely affected their daily lives. 11 patients were not able to walk unaided, though one patient walked without support but with considerable staggering, difficulties in half turn. Treatment with corticosteroids resulted in full recovery inTable 3 Efficacies of immunotherapies in patients with Hashimoto’s encephalopathyFull recovery Improvement Limited Sum improvement oral PSL mPSL mPSL + oral PSL 1 1 2 1 0 2 0 1 0 4 0 2 1 1 0 1 5 2 3 5 1 1 1mPSL + oral PSL + IS 0 mPSL + IVIg IVIg + IS Sum 0 0Improvement: patients started to walk without support and looked after their own affairs without help. Limited recovery: Unaided walking was still impossible. These data were based on published studies by Matsunaga et al. [58] and personal communication with the authors. mPSL intravenous methylprednisolone, oral PSL oral prednisolone, IVIg intravenous immunoglobulins, IS immunosuppressantsMitoma et al. Cerebellum Ataxias (2015) 2:Page 7 ofTable 4 Reported first line immunotherapy for each subtype Nelfinavir (Mesylate) of immune-mediated cerebellar ataxiasGluten ataxia Induction and maintenance therapies: strict gluten-free diet (In case of no improvement and negative gluten related antibodies, immunosuppressants or IVIg ) Paraneoplastic cerebellar degeneration Quick removal of neoplasm must be the first objective of treatment Induction therapy as soon as possible: mPSL, IVIg, immunosuppressants, or/and plasma exchange Discussion according associated Abs Maintenance therapy: continuous oral PSL, IVIg, immunosuppressants Anti-GAD Abs associated cerebellar ataxia Induction therapy: mPSL, IVIg, immunosuppressants, plasma exchange, or/and rituximab Maintenance therapy: continuous oral PSL, IVIg, immunosuppressants, or/and rituximabAbs antibodies, mPSL intravenous methylprednisolone, oral PSL oral prednisolone, IVIg intravenous immunoglobulinscould not walk unaided before treatment but limb ataxia improved slightly after IVIg, though unaided walking was still impossible. The above data do not favor IVIg relative to corticosteroids but rather suggest that corticosteroids should be considered first in the treatment. Excellent response to immunotherapy in HE correlated signifi.