Autoimmune post-herpes simplex encephalitis of adults and teenagers
Autoimmune post-herpes simplex encephalitis of adults and teenagers
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DOI：
10.1212/wnl.0000000000002125
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发表时间：
2015-11-17
期刊：
影响因子：
9.9
通讯作者：
Dalmau, Josep
中科院分区：
文献类型：
--
作者：
Armangue, Thais;Moris, German;Dalmau, Josep
目的：报道14例单纯疱疹病毒性脑炎(HSE)后免疫介导的复发症状，并比较青少年组和成人组与儿童组的临床和免疫学特征。方法：对2013年6月至2015年2月确诊的患者进行前瞻性观察研究。结果：在青少年和成人组(8名患者，中位年龄40岁，范围13-69岁；5名男性)中，3名患者有急性症状表现，提示病毒复发，5名患者连续出现HSE，提示既往缺陷复发。7名患者出现严重的精神/行为症状，扰乱了所有社交活动，1名患者出现难治性癫痫持续状态。1例患者出现眼睑痉挛。5例患者有抗NMDAR的脑脊液抗体，3例有抗未知神经细胞表面蛋白的抗体。在5/6的患者中，脑MRI显示新的对比剂强化区域，在免疫治疗和临床改善后减少。免疫治疗对7例患者有效，有时恢复良好，恢复到基线的HSE残留缺陷。与年龄较小的6名儿童(年龄中位数13个月，范围6-20，均带有NMDAR抗体)相比，青少年和成年人较少发生编舞运动(0/8vs6/6，p<；0.01)和意识水平下降(2/8vs6/6，p<；诊断和治疗延迟时间较长(间隔复发/抗体检测85天，17~296天，VS 4天，0~33天，P=0.037)。结论：青少年和成人HSE后的免疫介导性复发综合征不同于幼儿HSE后的舞蹈运动障碍，未被充分认识。及时诊断很重要，因为免疫疗法可以非常有效。
Objective:To report 14 patients with immune-mediated relapsing symptoms post-herpes simplex encephalitis (HSE) and to compare the clinical and immunologic features of the teenage and adult group with those of young children.Methods:Prospective observational study of patients diagnosed between June 2013 and February 2015. Immunologic techniques have been reported previously.Results:Among the teenage and adult group (8 patients, median age 40 years, range 13-69; 5 male), 3 had an acute symptom presentation suggesting a viral relapse, and 5 a presentation contiguous with HSE suggesting a recrudescence of previous deficits. Seven patients developed severe psychiatric/behavioral symptoms disrupting all social interactions, and one refractory status epilepticus. Blepharospasm occurred in one patient. Five patients had CSF antibodies against NMDA receptor (NMDAR) and 3 against unknown neuronal cell surface proteins. In 5/6 patients, the brain MRI showed new areas of contrast enhancement that decreased after immunotherapy and clinical improvement. Immunotherapy was useful in 7/7 patients, sometimes with impressive recoveries, returning to their baseline HSE residual deficits. Compared with the 6 younger children (median age 13 months, range 6-20, all with NMDAR antibodies), the teenagers and adults were less likely to develop choreoathetosis (0/8 vs 6/6, p < 0.01) and decreased level of consciousness (2/8 vs 6/6, p < 0.01) and had longer delays in diagnosis and treatment (interval relapse/antibody testing 85 days, range 17-296, vs 4 days, range 0-33, p = 0.037).Conclusion:In teenagers and adults, the immune-mediated relapsing syndrome post-HSE is different from that known in young children as choreoathetosis post-HSE and is underrecognized. Prompt diagnosis is important because immunotherapy can be highly effective.