Department of Neurology, EA2686, Université Lille Nord de France, Lille, France.
BMC Neurol. 2013 Dec 27;13:211. doi: 10.1186/1471-2377-13-211.
Encephalitis with anti-N-methyl-D-aspartate receptor antibodies (anti-NMDAR-Ab) is a rapid-onset encephalitis including psychosis, seizures, various movement disorders and autonomic system disturbances.
We report a very unusual case of extensive myelitis associated with anti-NMDAR-Ab. MRI also revealed a hyperintense T2 lesion, non-suggestive of MS, which progressively extended, associated with periventricular gadolinium enhancement visualized on brain MRI. Ophthalmological evaluation showed subclinical right optic neuritis. The absence of anti-AQP4 antibody argued against neuromyelitis optica spectrum disorder. A slight psychomotor slowing prompted us to search for various causes of autoimmune encephalitis. Anti-NMDAR-Ab was found in cerebrospinal fluid.
In patients with extensive myelitis who are seronegative for anti-AQP4 antibodies, and after other classical causes have been excluded, the hypothesis of atypical anti-NMDAR-Ab encephalitis should also be considered.
抗 N-甲基-D-天冬氨酸受体抗体（抗 NMDAR-Ab）脑炎是一种快速发作的脑炎，包括精神病、癫痫发作、各种运动障碍和自主神经系统紊乱。
我们报告了一例非常罕见的抗 NMDAR-Ab 相关广泛性脊髓炎病例。MRI 还显示 T2 高信号病变，不提示多发性硬化症，病变逐渐扩展，伴有脑 MRI 上可见的室周钆增强。眼科评估显示右侧视神经炎亚临床症状。抗 AQP4 抗体阴性排除了视神经脊髓炎谱系障碍。轻微的精神运动迟缓促使我们寻找各种自身免疫性脑炎的病因。脑脊液中发现了抗 NMDAR-Ab。
对于抗 AQP4 抗体阴性且已排除其他经典病因的广泛性脊髓炎患者，也应考虑不典型抗 NMDAR-Ab 脑炎的假说。
