Hara Makoto, Nakajima Hideto
Division of Neurology, Department of Medicine, Nihon University School of Medicine.
Brain Nerve. 2022 Oct;74(10):1163-1170. doi: 10.11477/mf.1416202204.
Autoimmune disorders, such as choreoathetosis and abnormal behavior secondary to herpes simplex encephalitis (HSE), are discussed in this review. These disorders are known to develop without any evidence of HSE relapse, while they respond to immunotherapies such as intravenous corticosteroids. Recent evidence, including a prospective Spanish cohort study of HSE, revealed that autoimmune encephalitis (AE) can be triggered by HSE, which is closely related to several neuronal surface antibodies (NSAs). Anti-N-methyl-D-aspartate encephalitis (NMDARE) is the most common phenotype of AE post-HSE. Moreover, approximately 30% of cases of AE post-HSE are caused by NSAs against undetermined antigens. Thus, patients suspected of having AE post-HSE should be tested for NSAs using comprehensive techniques combining tissue-based and cultured live neuron assays. The primary syndrome of AE post-HSE is age dependent, as demonstrated in non-post-HSE onset NMDARE. Choreoathetosis is the most common symptom in infants and toddlers, while abnormal behavior and psychiatric symptoms are the most common symptoms in adolescents and adults. Regarding the treatment, current knowledge reveals that intensive immunotherapies can be used to treat AE post-HSE and lead to better outcomes although a minority of cases show recovery without immunotherapy administration.
本综述讨论了自身免疫性疾病,如继发于单纯疱疹病毒性脑炎(HSE)的舞蹈徐动症和异常行为。已知这些疾病在无HSE复发证据的情况下发生,而它们对免疫疗法(如静脉注射皮质类固醇)有反应。最近的证据,包括一项对HSE的前瞻性西班牙队列研究,表明自身免疫性脑炎(AE)可由HSE引发,这与几种神经元表面抗体(NSA)密切相关。抗N-甲基-D-天冬氨酸脑炎(NMDARE)是HSE后AE最常见的表型。此外,约30%的HSE后AE病例由针对未确定抗原的NSA引起。因此,怀疑患有HSE后AE的患者应使用结合基于组织和培养活神经元检测的综合技术检测NSA。HSE后AE的主要综合征与年龄有关,这在非HSE后发病的NMDARE中得到了证实。舞蹈徐动症是婴幼儿最常见的症状,而异常行为和精神症状是青少年和成年人最常见的症状。关于治疗,目前的知识表明,强化免疫疗法可用于治疗HSE后AE并带来更好的结果,尽管少数病例在未进行免疫治疗的情况下也会康复。