Rvival time from beginning of immunotherapy was 10.2 months (range, 2?8) and 14 patients

Rvival time from beginning of immunotherapy was 10.2 months (range, 2?8) and 14 patients died during follow-up (88 ). Of these, death was cancerrelated in 9 patients, neurological disease-related in 3, and unknown reason in 2 patients (suicide? or unknown). Thus, at the end of follow-up, only 2 patients were alive. Taken together, these long-term follow-up studies suggest that PCD with Hu or Yo-Abs are associated with severe CAs resistant to immunotherapy (including combination therapies). Apart from the above long-term follow-up studies, several other retrospective studies and case reports have suggested the beneficial effects of corticosteroids and IVIg in patients with anti-Hu or anti-Yo antibodies (Additional file 2: Table S2) [25?1]. Among 9 responders, 1 patient received mPSL + cyclophosphamide, 2 patients IVIg, 1 patient IVIg + plasma exchange, 3 patients cyclophosphamide, 1 patient IVIg + oral prednisolone (PSL) + cyclophosphamide, and 1 patient IVIg + oral PSL + rituximab. The mean follow-up period ranged from 4 months to 10 years (median 33.9 months). Of these studies, Voltz [20] proposed a trial of immunotherapies after cancer treatment in patients with PCD. Specifically, the treatment regimen comprises one course of mPSL, followed 1 or 2 weeks later in case of lack of response by administration of IVIg. Lack of response to IVIg should be followed 1 or 2 weeks later by plasma exchange or cyclophosphamide.Prognostic factorsThere are no specific studies on the prognosis of PCD according to associated autoantibodies. However, prognosis seems to vary depending on the nature of the associated ONA. In some cases, such as PCD with Yo-Abs, cerebellar ataxia is clearly due to Purkinje cell death and neurological improvement is unlikely. The objective of treatment will be to stop the immune process resulting in neuronal death. In other cases, a neuronal blockage can be suspected before secondary neuronal death occurs and in these cases, immunomodulator treatment and tumor removal become an emergency. For example, anti-Tr/DNER-associated PCD is particularly remarkable for the improvement observed after efficient treatment of the associated lymphoma, irrespective of additional immunotherapy [32]. Furthermore, some patients with anti-Ri Ab also seem to respond well to immunotherapy [33]. This is by contrast to the poor prognosis of PCD with Yo, Hu or CV2/CRMP5-Abs suggesting different pathological mechanisms for each subtypes Nelfinavir (Mesylate) of paraneoplastic antibodies [34]. Finally some authors suggestedMitoma et al. Cerebellum Ataxias (2015) 2:Page 5 ofthat early introduction of immunotherapy could PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/16989806 be associated with better outcome [20]. The data demonstrate the need for future prospective studies comparing patient outcomes and immunomodulator treatment responses based on associated autoantibodies [35].Anti-GAD Abs associated cerebellar ataxia What is the first line immunotherapy?Although various types of immunotherapies have been used in patients with GAD Abs-CA, most patients develop significant disability with poor prognosis [1, 7].Short-term follow-upTable 2 summarizes the effects of induction therapies in 20 patients with short-term follow-up [36?0] (see also Additional file 3: Table S3). Classification of patients into two types (subacute and chronic types) demonstrated good response to immunotherapy in patients with subacute type. The onset of CAs was defined as subacute when CAs reached their NADIR or required neurological assessment within.

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