Mittal Manoj K, Rabinstein Alejandro A, Hocker Sara E, Pittock Sean J, Wijdicks Eelco F M, McKeon Andrew
Department of Neurology, College of Medicine, Mayo Clinic, 200 First Street S.W., Rochester, MN, 55905, USA.
Department of Neurology, University of Kansas Medical Center, Kansas, KS, USA.
Neurocrit Care. 2016 Apr;24(2):240-50. doi: 10.1007/s12028-015-0196-8.
To report the clinical and laboratory characteristics, clinical courses, and outcomes of Mayo Clinic, Rochester, MN, ICU-managed autoimmune encephalitis patients (January 1st 2003-December 31st 2012).
Based on medical record review, twenty-five patients were assigned to Group 1 (had ≥1 of classic autoimmune encephalitis-specific IgGs, n = 13) or Group 2 (had ≥3 other characteristics supporting autoimmunity, n = 12).
Median admission age was 47 years (range 22-88); 17 were women. Initial symptoms included ≥1 of subacute confusion or cognitive decline, 13; seizures, 12; craniocervical pain, 5; and personality change, 4. Thirteen Group 1 patients were seropositive for ≥1 of VGKC-complex-IgG (6; including Lgi1-IgG in 2), NMDA-R-IgG (4), AMPA-R-IgG (1), ANNA-1 (1), Ma1/Ma2 antibody (1), and PCA-1 (1). Twelve Group 2 patients had ≥3 other findings supportive of an autoimmune diagnosis (median 4; range 3-5): ≥1 other antibody type detected, 9; an inflammatory CSF, 8; ≥1 coexisting autoimmune disease, 7; an immunotherapy response, 7; limbic encephalitic MRI changes, 5; a paraneoplastic cause, 4; and diagnostic neuropathological findings, 2. Among 11 patients ICU-managed for ≥4 days, neurological improvements were attributable to corticosteroids (5/7 treated), plasmapheresis (3/7), or rituximab (1/3). At last follow-up, 10 patients had died. Of the remaining 15 patients, 6 (24%) had mild or no disability, 3 (12%) had moderate cognitive problems, and 6 (24%) had dementia (1 was bed bound). Median modified Rankin score at last follow-up was 3 (range 0-6).
Good outcomes may occur in ICU-managed autoimmune encephalitis patients. Clinical and testing characteristics are diverse. Comprehensive diagnostics should be pursued to facilitate timely treatment.
报告明尼苏达州罗切斯特市梅奥诊所重症监护病房（ICU）管理的自身免疫性脑炎患者（2003年1月1日至2012年12月31日）的临床和实验室特征、临床病程及转归。
基于病历回顾，25例患者被分为第1组（具有≥1种经典自身免疫性脑炎特异性IgG，n = 13）或第2组（具有≥3种支持自身免疫的其他特征，n = 12）。
入院年龄中位数为47岁（范围22 - 88岁）；17例为女性。初始症状包括亚急性意识模糊或认知功能减退（13例）、癫痫发作（12例）、颅颈疼痛（5例）及人格改变（4例）中的≥1种。第1组13例患者中，1种或以上电压门控性钾通道复合物（VGKC）-IgG（6例；包括2例富含亮氨酸胶质瘤失活蛋白1（LGI1）-IgG）、N-甲基-D-天冬氨酸受体（NMDA-R）-IgG（4例）、α-氨基-3-羟基-5-甲基-4-异恶唑丙酸受体（AMPA-R）-IgG（1例）、抗Hu抗体（ANNA-1，1例）、抗Ma1/Ma2抗体（1例）及抗Yo抗体（PCA-1，1例）检测呈阳性。第2组12例患者具有≥3种支持自身免疫诊断的其他发现（中位数4项；范围3 - 5项）：检测到≥1种其他抗体类型（9例）、脑脊液炎性改变（8例）、≥1种并存的自身免疫性疾病（7例）、免疫治疗反应（7例）、边缘叶脑炎的磁共振成像（MRI）改变（5例）、副肿瘤病因（4例）及诊断性神经病理学发现（2例）。在11例在ICU管理≥4天的患者中，神经功能改善归因于皮质类固醇（7例中5例接受治疗）、血浆置换（7例中3例）或利妥昔单抗（3例中1例）。在末次随访时，10例患者死亡。其余15例患者中，6例（24%）有轻度残疾或无残疾，3例（12%）有中度认知问题，6例（24%）有痴呆（1例卧床）。末次随访时改良Rankin评分中位数为3分（范围0 - 6分）。
ICU管理的自身免疫性脑炎患者可能有良好转归。临床和检测特征多样。应进行全面诊断以便及时治疗。
