Retrospective analysis of NMDA receptor antibodies in encephalitis of unknown origin

Retrospective analysis of NMDA receptor antibodies in encephalitis of unknown origin
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DOI:
10.1212/wnl.0b013e3181fc2a06
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发表时间:
2010-11-09
期刊:
影响因子:
9.9
通讯作者:
Wandinger, K. P.
Wandinger, K. P.
中科院分区:
医学1区
文献类型:
--
作者:
Pruess, H.;Dalmau, J.;Wandinger, K. P.

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背景资料:抗NMDA受体(NMDAR)脑炎是一种严重的疾病,与NMDAR NR 1亚单位抗体有关,并导致一种特征性的综合征。目的:确定在一个单一的机构中,既往诊断为不明原因脑炎的患者是否患有抗NMDAR脑炎。回顾性分析了5年期间入住重症监护室(ICU)的505例年龄在18岁至35岁之间的患者的病历,以确定病因不明的脑炎标准。这些包括脑炎体征伴精神症状(激越、偏执想法、易怒或幻觉);癫痫发作; CSF炎症;排除病毒或细菌感染。检查符合这些标准的患者的存档血清和CSF样品的NMDAR抗体。随访访问允许的自然病程的分析和预后的估计。结果:7例患者(所有妇女)符合指定的标准,其中6人有NMDAR抗体。卵巢畸胎瘤检出2例,其中1例发生在脑炎后3年。所有患者的结局均良好。一名没有畸胎瘤的患者随着NMDAR抗体的消失而沿着自发改善。结论:抗NMDAR脑炎占所有入住ICU的年轻患者的1%。7例符合临床标准的病例中有6例患有抗NMDAR脑炎。应在所有符合这些标准的脑炎患者中检测NMDAR抗体。神经病学(R)2010;75:1735-1739
Background: Anti-NMDA-receptor (NMDAR) encephalitis is a severe disorder that occurs in association with antibodies to the NR1 subunit of the NMDAR and results in a characteristic syndrome.Objective: To determine in a single institution setting whether patients previously diagnosed with encephalitis of unknown origin had anti-NMDAR encephalitis.Methods: Charts of 505 patients aged 18 to 35 years admitted to the intensive care unit (ICU) during a 5-year period were retrospectively reviewed for criteria of encephalitis of unknown etiology. These included encephalitic signs with psychiatric symptoms (agitation, paranoid thoughts, irritability, or hallucinations); seizures; CSF inflammation; and exclusion of viral or bacterial infection. Archived serum and CSF samples of patients fulfilling these criteria were examined for NMDAR antibodies. Follow-up visits allowed the analysis of the natural disease course and estimation of prognosis.Results: Seven patients (all women) fulfilled the indicated criteria; 6 of them had NMDAR antibodies. Ovarian teratomas were detected in 2 patients, in one 3 years after the onset of encephalitis. Outcome was favorable in all patients. One patient without teratoma improved spontaneously along with disappearance of NMDAR antibodies.Conclusions: Anti-NMDAR encephalitis represented 1% of all young patients' admissions to the ICU. Six of 7 cases with the indicated clinical criteria had anti-NMDAR encephalitis. NMDAR antibodies should be tested in all patients with encephalitis who fulfill these criteria. Neurology(R) 2010;75:1735-1739