Department of Neurology, Qilu Hospital of Shandong University, Jinan, 250012, Shandong, China.
Department of Neurology, Shandong Provincial Qianfoshan Hospital, Shandong University, Jinan, 250014, Shandong, China.
Cerebellum. 2024 Aug;23(4):1697-1701. doi: 10.1007/s12311-023-01636-z. Epub 2023 Nov 22.
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.
抗 DNER 抗体与副肿瘤性小脑变性（PCD）和霍奇金病（HD）有关。然而，最近的研究报道了一些病例缺乏 HD，并且非肿瘤性抗 DNER 抗体相关的共济失调特征不明显。我们报告了一例伴有抗 DNER 抗体的急性小脑共济失调和眼球震颤。脑部磁共振成像（MRI）显示小脑萎缩。在 CSF 和血清中检测到高滴度的抗 DNER 抗体。在 10 个月的随访中，正电子发射断层扫描（PET）扫描无明显异常。患者在接受静脉注射类固醇、免疫球蛋白和利妥昔单抗的免疫抑制治疗后显著改善。我们的研究表明，存在这种抗神经元抗体可能与恶性肿瘤无关，并且早期发病的非肿瘤患者在免疫治疗后更有可能获得更好的结果。早期诊断和及时的免疫抑制治疗可能对这些患者有益。
