Ann Neurol. 2014 Mar;75(3):411-28. doi: 10.1002/ana.24117.
To report the clinical, radiological, and immunological association of demyelinating disorders with anti–Nmethyl- D-aspartate receptor (NMDAR) encephalitis.
Clinical and radiological analysis was done of a cohort of 691 patients with anti-NMDAR encephalitis. Determination of antibodies to NMDAR, aquaporin-4 (AQP4), and myelin oligodendrocyte glycoprotein (MOG) was performed using brain immunohistochemistry and cell-based assays.
Twenty-three of 691 patients with anti-NMDAR encephalitis had prominent magnetic resonance imaging (MRI) and/or clinical features of demyelination. Group 1 included 12 patients in whom anti-NMDAR encephalitis was preceded or followed by independent episodes of neuromyelitis optica (NMO) spectrum disorder (5 cases, 4 anti-AQP4 positive) or brainstem or multifocal demyelinating syndromes (7 cases, all anti-MOG positive). Group 2 included 11 patients in whom anti-NMDAR encephalitis occurred simultaneously with MRI and symptoms compatible with demyelination (5 AQ4 positive, 2 MOG positive). Group 3 (136 controls) included 50 randomly selected patients with typical anti-NMDAR encephalitis, 56 with NMO, and 30 with multiple sclerosis; NMDAR antibodies were detected only in the 50 anti-NMDAR patients, MOG antibodies in 3 of 50 anti-NMDAR and 1 of 56 NMO patients, and AQP4 antibodies in 48 of 56 NMO and 1 of 50 anti-NMDAR patients (p<0.0001 for all comparisons with Groups 1 and 2). Most patients improved with immunotherapy, but compared with anti-NMDAR encephalitis the demyelinating episodes required more intensive therapy and resulted in more residual deficits. Only 1 of 23 NMDAR patients with signs of demyelination had ovarian teratoma compared with 18 of 50 anti-NMDAR controls (p50.011).
Patients with anti-NMDAR encephalitis may develop concurrent or separate episodes of demyelinating disorders, and conversely patients with NMO or demyelinating disorders with atypical symptoms (eg, dyskinesias, psychosis) may have anti-NMDAR encephalitis.
报告抗 N-甲基-D-天冬氨酸受体（NMDAR）脑炎伴发脱髓鞘疾病的临床、影像学和免疫学关联。
对 691 例抗 NMDAR 脑炎患者进行临床和影像学分析。采用脑免疫组化和细胞检测法测定抗 NMDAR、水通道蛋白-4（AQP4）和髓鞘少突胶质细胞糖蛋白（MOG）抗体。
691 例抗 NMDAR 脑炎患者中有 23 例有突出的磁共振成像（MRI）和/或脱髓鞘的临床特征。第 1 组包括 12 例患者，他们的抗 NMDAR 脑炎之前或之后有视神经脊髓炎（NMO）谱系障碍（5 例，4 例抗 AQP4 阳性）或脑干或多灶性脱髓鞘综合征（7 例，均抗 MOG 阳性）的独立发作。第 2 组包括 11 例患者，他们的抗 NMDAR 脑炎同时伴有 MRI 和与脱髓鞘相符的症状（5 例 AQP4 阳性，2 例 MOG 阳性）。第 3 组（对照组 136 例）包括 50 例随机选择的典型抗 NMDAR 脑炎患者、56 例 NMO 患者和 30 例多发性硬化症患者；仅在 50 例抗 NMDAR 患者中检测到 NMDAR 抗体，在 50 例抗 NMDAR 患者中的 3 例和 56 例 NMO 患者中的 1 例检测到 MOG 抗体，在 56 例 NMO 患者中的 48 例和 50 例抗 NMDAR 患者中的 1 例检测到 AQP4 抗体（与第 1 组和第 2 组相比，所有比较均为 p<0.0001）。大多数患者经免疫治疗后好转，但与抗 NMDAR 脑炎相比，脱髓鞘发作需要更强化的治疗，导致更多的残留缺陷。与 50 例抗 NMDAR 对照相比，仅 23 例有脱髓鞘迹象的 NMDAR 患者中有 1 例有卵巢畸胎瘤（p50.011）。
抗 NMDAR 脑炎患者可能会同时或分别发生脱髓鞘疾病，反之，NMO 或有非典型症状（如运动障碍、精神病）的脱髓鞘疾病患者可能患有抗 NMDAR 脑炎。
