一名患有与N-甲基-D-天冬氨酸受体抗体相关脑炎的患者。
A patient with encephalitis associated with NMDA receptor antibodies.
作者信息
Sansing Lauren H, Tüzün Erdem, Ko Melissa W, Baccon Jennifer, Lynch David R, Dalmau Josep
机构信息
Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
出版信息
Nat Clin Pract Neurol. 2007 May;3(5):291-6. doi: 10.1038/ncpneuro0493.
BACKGROUND
A 34-year-old woman presented with headache, feverish sensation and anxiety, rapidly followed by homicidal ideation, aggressive agitation, seizures, hypoventilation, hyperthermia and prominent autonomic instability requiring intubation and sedation. She developed episodes of hypotension and bradycardia with periods of asystole lasting up to 15 seconds. Upon weaning off sedation, her eyes opened but she was unresponsive to stimuli. There was muscle rigidity, frequent facial grimacing, rhythmic abdominal contractions, kicking motions of the legs, and intermittent dystonic postures of the right arm.
INVESTIGATIONS
Routine laboratory testing, toxicology screening, studies for autoimmune and infectious etiologies, brain MRI scan, lumbar puncture, electroencephalogram, whole-body CT scan, abdominal ultrasound, paraneoplastic and voltage-gated potassium channel antibody serologies, analysis of N-methyl-D-aspartate receptor antibodies.
DIAGNOSIS
Paraneoplastic encephalitis associated with immature teratoma of the ovary and N-methyl-D-aspartate receptor antibodies.
MANAGEMENT
Intensive care, mechanical ventilation, antiepileptics, laparotomy and left salpingo-oophorectomy, corticosteroids, plasma exchange, intravenous immunoglobulin, cyclophosphamide, physical therapy, and chemotherapy.
背景
一名34岁女性出现头痛、发热感和焦虑症状，随后迅速出现杀人念头、攻击性躁动、癫痫发作、通气不足、体温过高以及明显的自主神经功能不稳定，需要插管和镇静。她出现了低血压和心动过缓发作，伴有长达15秒的心脏停搏期。在停用镇静剂后，她的眼睛睁开，但对刺激无反应。存在肌肉僵硬、频繁面部怪相、有节律的腹部收缩、腿部踢动动作以及右臂间歇性张力障碍姿势。
检查
常规实验室检查、毒理学筛查、自身免疫和感染病因学研究、脑部磁共振成像扫描、腰椎穿刺、脑电图、全身计算机断层扫描、腹部超声、副肿瘤和电压门控钾通道抗体血清学检查、N-甲基-D-天冬氨酸受体抗体分析。
诊断
与卵巢未成熟畸胎瘤和N-甲基-D-天冬氨酸受体抗体相关的副肿瘤性脑炎。
治疗
重症监护、机械通气、抗癫痫药物、剖腹手术和左侧输卵管卵巢切除术、皮质类固醇、血浆置换、静脉注射免疫球蛋白、环磷酰胺、物理治疗和化疗。
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