From the Chulalongkorn Center of Excellence on Parkinson's Disease and Related Disorders (P.J., R.B.), Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Thai Red Cross Society, Bangkok, Thailand; University of Oxford (A.V.), UK; and the Geffen School of Medicine at UCLA (R.B.), Los Angeles, CA.
Neurology. 2014 May 6;82(18):1662-3. doi: 10.1212/WNL.0000000000000384. Epub 2014 Apr 4.
A confused and agitated 18-year-old woman presented to the emergency unit with orolingual movements, eye deviation, and a temperature of 38°C. The symptoms had begun 2 weeks prior to the admission when she developed a severe headache associated with pathologic laughing and intermittent episodes of upgaze deviation. A urine pregnancy test was positive and a transvaginal ultrasonography showed a 9-week-old fetus. An MRI of the brain was unremarkable and results of the CSF analysis were also unremarkable apart from a CSF pleocytosis (62 lymphocytes) and slightly elevated protein (55 mg/dL; normal range 0-45 mg/dL). Extensive microbiologic and serologic studies with CSF were all negative. She gradually lost consciousness, experienced respiratory failure, and was intubated. There were semirhythmic movements consisting of complex patterns of mouth opening, chewing, facial grimacing, synchronous flexion-extension, and supination-pronation limb movements, which persisted during the period of unresponsiveness. She also had generalized hyperreflexia, persistent hyperthermia, and a full bladder. Three EEGs showed diffuse slow waves with no epileptic discharges. A diagnosis of anti-NMDA receptor (NMDAR) encephalitis was made on clinical grounds and strongly positive serum NMDAR antibodies.
一位 18 岁的年轻女性，表现出口周运动、眼球偏斜和 38°C 的发热，伴意识混乱和激越，前来急诊就诊。这些症状在入院前 2 周开始出现，当时她出现严重头痛，伴有病理性大笑和间歇性上视障碍。尿液妊娠试验阳性，经阴道超声检查显示为 9 周大的胎儿。脑部 MRI 未见异常，脑脊液分析结果也未见异常，除了脑脊液白细胞增多（62 个淋巴细胞）和略微升高的蛋白（55mg/dL；正常值 0-45mg/dL）。广泛的微生物和血清学研究与脑脊液均为阴性。她逐渐失去意识，发生呼吸衰竭，并被插管。在无反应期间，她出现了半节律性运动，包括口部张开、咀嚼、面部鬼脸、同步屈伸和旋前旋后肢体运动的复杂模式。她还出现全身性反射亢进、持续高热和膀胱充盈。三次脑电图显示弥漫性慢波，无癫痫放电。根据临床特征和强烈阳性的血清 NMDA 受体（NMDAR）抗体，诊断为抗 NMDA 受体（NMDAR）脑炎。
