Abe Keith K, Koli Roshni L, Yamamoto Loren G
From the Department of *Pediatrics and †Psychiatry, John A. Burns School of Medicine, University of Hawaii, Honolulu, HI.
Pediatr Emerg Care. 2016 Feb;32(2):107-12; quiz 113-5. doi: 10.1097/PEC.0000000000000713.
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an acute autoimmune neurological disorder that presents with acute to subacute psychiatric and/or neurological complaints including new onset behavioral changes that may evolve to psychosis and catatonia, cognitive decline, new onset seizures, progressive encephalopathy, and/or movement disorders. Female teens and adults often have an associated ovarian teratoma as an underlying etiology, but most pediatric patients do not have an identifiable associated neoplasm. The diagnosis requires confirmatory serum and/or cerebrospinal fluid analysis findings of anti-NMDAR antibody titers. It can be misdiagnosed as a psychiatric condition or a viral encephalitis. The clinical features that distinguish anti-NMDAR encephalitis from a primary psychiatric disorder are the acute onset of the mood and behavioral changes with no history, the waxing and waning of consciousness (delirium/encephalopathy), and primary neurological features such as seizures and abnormal involuntary movements, including dyskinesias and dystonias. The prognosis is improved with earlier recognition and prompt immunotherapy treatment, making this an important diagnosis for emergency physicians.
抗N-甲基-D-天冬氨酸受体(抗NMDAR)脑炎是一种急性自身免疫性神经疾病,表现为急性至亚急性的精神和/或神经症状,包括新发的行为改变,可能发展为精神病和紧张症、认知衰退、新发癫痫、进行性脑病和/或运动障碍。青少年女性和成年女性常伴有卵巢畸胎瘤作为潜在病因,但大多数儿科患者没有可识别的相关肿瘤。诊断需要抗NMDAR抗体滴度的血清和/或脑脊液分析结果来确诊。它可能被误诊为精神疾病或病毒性脑炎。抗NMDAR脑炎与原发性精神障碍相区别的临床特征是情绪和行为改变急性起病且无病史、意识波动(谵妄/脑病)以及癫痫和异常不自主运动等原发性神经特征,包括运动障碍和肌张力障碍。早期识别并及时进行免疫治疗可改善预后,这使其成为急诊医生的重要诊断疾病。