Matoq Amr A, Rappoport Adam S, Yang Yiting, O'Babatunde Jessica, Bakerywala Rubina, Sheth Raj D
University of Florida & Wolfson Children Hospital, Jacksonville, FL, USA.
Nemours Children Health System, Jacksonville, FL, USA; Mayo Clinic, Jacksonville, FL, USA.
Epilepsy Behav Case Rep. 2015 Nov 4;4:99-101. doi: 10.1016/j.ebcr.2015.07.005. eCollection 2015.
Anti-N-methyl-d-aspartate (NMDA) receptor antibody encephalitis is an autoimmune disorder manifesting subacutely with prominent aberrant movements and psychiatric symptoms. The clinical course is one of progressive clinical deterioration that can be halted and often reversed by early diagnosis and treatment. Patterns of presentation and etiology of anti-NMDA-receptor antibody encephalitis are dependent on age and can be challenging to recognize in very young children.
Sequential clinical case observations of anti-NMDA-receptor antibody encephalitis presenting in very young children were examined over a year at a single tertiary pediatric institution. Cerebrospinal fluid confirmed anti-NMDA-receptor antibodies in two cases (a 21-month-old boy and a 29-month-old girl) that demonstrated either bizarre behavioral patterns or status epilepticus both associated with progressive deterioration. Once recognized, the clinical course was arrested and reversed by aggressive treatment with plasma exchange, immunoglobulin, and high dose IV steroids.
Infants with anti-NMDA-receptor antibody encephalitis can present with frank seizures or seizure mimics. Regardless, prompt recognition and aggressive treatment of anti-NMDA-receptor antibody encephalitis, while challenging, can quickly arrest deterioration and hasten recovery, thereby, limiting neurological morbidity.
抗N-甲基-D-天冬氨酸（NMDA）受体抗体脑炎是一种自身免疫性疾病，亚急性起病，伴有明显的异常运动和精神症状。其临床病程呈进行性临床恶化，早期诊断和治疗可使其停止并常能逆转。抗NMDA受体抗体脑炎的表现形式和病因取决于年龄，在幼儿中识别可能具有挑战性。
在一家三级儿科机构对1年内幼儿期抗NMDA受体抗体脑炎的连续临床病例观察进行了研究。脑脊液证实两例（一名21个月大男孩和一名29个月大女孩）存在抗NMDA受体抗体，这两例均表现出怪异行为模式或癫痫持续状态，均与病情进展恶化相关。一旦确诊，通过血浆置换、免疫球蛋白和大剂量静脉注射类固醇积极治疗，临床病程得以停止并逆转。
抗NMDA受体抗体脑炎患儿可出现明显癫痫发作或类似癫痫发作的症状。无论如何，尽管具有挑战性，但对抗NMDA受体抗体脑炎的及时识别和积极治疗可迅速阻止病情恶化并加速康复，从而限制神经功能损害。
