Neurology Unit, Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Italy.
Departments of Laboratory Medicine and Pathology, Rochester, MN, USA; Neurology Mayo Clinic, Rochester, MN, USA.
J Neuroimmunol. 2022 Jul 15;368:577882. doi: 10.1016/j.jneuroim.2022.577882. Epub 2022 May 5.
A 67-years-old woman developed subacute oculomotor nerve palsy and cerebellar gait instability while receiving avelumab as immunotherapy for Merkel cell carcinoma. Brain MRI revealed oculomotor nerve T2/FLAIR hyperintensity and contrast enhancement, CSF cell number and protein concentration were slightly increased. Antibodies against intracellular and surface antigens were excluded through commercial assays, but home-made immunohistochemistry on rat brain sections showed a "neurofilament-like" pattern. Antibodies against neuronal intermediate filament (NIF-IgG) were thus tested and resulted positive in both serum and CSF, confirming the diagnosis of NIF-IgG autoimmunity. Avelumab was discontinued and treatment with steroids and intravenous immunoglobulins led to partial improvement.
一位 67 岁女性在接受 Merkel 细胞癌的免疫治疗用avelumab 时，出现亚急性动眼神经麻痹和小脑步态不稳。脑部 MRI 显示动眼神经 T2/FLAIR 信号增高和对比增强，CSF 细胞数和蛋白浓度略有增加。通过商业检测排除了针对细胞内和细胞表面抗原的抗体，但在大鼠脑切片上进行的自制免疫组化显示出“神经丝样”模式。针对神经元中间丝（NIF-IgG）的抗体进行了检测，血清和 CSF 均为阳性，确诊为 NIF-IgG 自身免疫。停用 avelumab，并采用类固醇和静脉注射免疫球蛋白治疗，部分改善。
