De Maeseneire Charlotte, Tahry Riem El, Santos Susana Ferrao
Cliniques Universitaires Saint-Luc - Neurology Department, Bruxelles, Belgium.
Epileptic Disord. 2017 Dec 1;19(4):471-475. doi: 10.1684/epd.2017.0945.
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder of the central nervous system that typically manifests predominantly as a psychiatric disorder. However, other manifestations such as epileptic seizures, abnormal movements, and memory or language complications are not unusual. Here, we report the case of a young man who presented with a new-onset epilepsy, with ictal semiology suggestive of insular involvement; this hypothesis was supported by a PET-CT study. Anti-NMDAR antibodies were found in the CSF, confirming the diagnosis of anti-NMDAR encephalitis. A review of the literature reveals that epilepsy can be the first manifestation of NMDAR encephalitis, with a clear male predominance. Despite its rarity, neurologists should consider this diagnosis for any young patient developing a new-onset epilepsy with temporal or insular features, particularly if the patient is male. Other cognitive or behavioural signs, even very subtle, should also prompt diagnosis.
抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎是一种中枢神经系统自身免疫性疾病，通常主要表现为精神障碍。然而，癫痫发作、异常运动以及记忆或语言方面的并发症等其他表现也并不罕见。在此，我们报告一例年轻男性病例，该患者新发癫痫，发作期症状学提示岛叶受累；这一假说得到了PET-CT研究的支持。脑脊液中发现了抗NMDAR抗体，确诊为抗NMDAR脑炎。文献回顾显示，癫痫可能是NMDAR脑炎的首发表现，男性明显居多。尽管该病罕见，但对于任何出现具有颞叶或岛叶特征的新发癫痫的年轻患者，尤其是男性患者，神经科医生都应考虑这一诊断。其他认知或行为体征，即使非常细微，也应促使进行诊断。
