A patient with encephalitis associated with NMDA receptor antibodies
A patient with encephalitis associated with NMDA receptor antibodies
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DOI：
10.1038/ncpneuro0493
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发表时间：
2007-05-01
期刊：
影响因子：
--
通讯作者：
Dalmau, Josep
中科院分区：
文献类型：
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作者：
Sansing, Lauren H.;Tuzun, Erdem;Dalmau, Josep
背景：一位34岁女性，以头痛、发热、焦虑为主诉，随后迅速出现杀人意念、攻击性激越、癫痫发作、低通气、体温过高和明显的自主神经不稳定，需要插管和镇静。她出现低血压和心动过缓发作，心搏停止持续长达15秒。停用镇静剂后，她的眼睛睁开，但对刺激无反应。常规实验室检查、毒理学筛查、自身免疫性和感染性病因学研究、脑MRI扫描、腰椎穿刺、脑电图、全身CT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、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。
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.