Wickström Ronny, Fowler Asa, Cooray Gerald, Karlsson-Parra Alex, Grillner Pernilla
Neuropediatric Unit, Department of Women's and Children's Health, Karolinska Institutet, Sweden.
Neuropediatric Unit, Department of Women's and Children's Health, Karolinska Institutet, Sweden.
Eur J Paediatr Neurol. 2014 Jul;18(4):543-6. doi: 10.1016/j.ejpn.2014.03.011. Epub 2014 Apr 8.
Herpes simplex encephalitis (HSE) in children is a potentially devastating condition which is occasionally complicated by a clinical relapse. An autoimmune component has long been suspected in these relapses and recent findings suggest that antibodies against N-methyl-D-aspartate receptors (NMDARs) may be part of this mechanism. We here report an 11 months old girl with acute HSE and with negative NMDAR antibody serology at presentation who after an initial response to antiviral treatment deteriorated with seizures, abnormal movements, focal neurologic deficits and psychiatric symptoms. We show that this relapse occurred as production of NMDAR antibodies developed and that clinical improvement followed immunotherapy with a concomitant decrease in NMDAR antibody titers in CSF. She also developed a characteristic 15-20 Hz activity over both hemispheres which has been previously described as an electroencephalographic presentation of anti-NMDAR encephalitis. We conclude that relapse or persisting symptoms in HSE in children may represent an immune-mediated mechanism rather than a viral reactivation and that NMDAR antibodies should be analyzed as this may be of importance for the choice of therapy.
儿童单纯疱疹病毒性脑炎（HSE）是一种潜在的严重疾病，偶尔会出现临床复发。长期以来，人们一直怀疑这些复发存在自身免疫成分，最近的研究结果表明，针对N-甲基-D-天冬氨酸受体（NMDARs）的抗体可能是这一机制的一部分。我们在此报告一名11个月大的女童，她患有急性HSE，就诊时NMDAR抗体血清学检测呈阴性，在对抗病毒治疗产生初始反应后，病情恶化，出现癫痫发作、异常运动、局灶性神经功能缺损和精神症状。我们发现，此次复发发生在NMDAR抗体产生之时，免疫治疗后临床症状改善，同时脑脊液中NMDAR抗体滴度降低。她还在双侧半球出现了特征性的15 - 20 Hz活动，此前曾被描述为抗NMDAR脑炎的脑电图表现。我们得出结论，儿童HSE的复发或持续症状可能代表一种免疫介导机制，而非病毒再激活，应分析NMDAR抗体，因为这可能对治疗方案的选择具有重要意义。
