Abstract
Anti-DNER antibody is associated with paraneoplastic cerebellar degeneration (PCD) and Hodgkin’s disease (HD). However, recent studies reported cases absence of HD and that non-tumor anti-DNER antibody-associated ataxia was not well characterized. We present a case of acute cerebellar ataxia and nystagmus with detected anti-DNER antibody. Brain magnetic resonance imaging (MRI) showed cerebellar atrophy. High titer of anti-DNER antibody was detected in CSF and serum. Positron emission tomography (PET) scanning was unremarkable at a 10-month follow up. The patient improved significantly after immunosuppressive therapy with intravenous steroids, immunoglobulin followed by rituximab. Our study suggest that the presence of such anti-neuronal antibodies might not come along with malignancy and that early onset non-tumor patients are more likely to have a better outcome after immunotherapy. Early diagnosis and timely immunosuppressive therapy may prove beneficial for these patients.
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Funding
This work is supported by National Natural Science Foundation of China (No. 82101487), Natural Science Foundation of Shandong Province, China (ZR2021QH161) and Taishan Scholar Program of Shandong Province (tsqn202211318).
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RN.D and WY.S wrote the main manuscript. RN.D prepared figure 1 and 2. All authors reviewed the manuscript.
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Duan, RN., Si, WY. & Cao, LL. Favorable Outcomes in a Case of Non-paraneoplastic DNER Ataxia Treated with Immunotherapy. Cerebellum (2023). https://doi.org/10.1007/s12311-023-01636-z
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DOI: https://doi.org/10.1007/s12311-023-01636-z