Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu 610041, China; Key Laboratory of Obstetrics & Gynecologic and Pediatric Diseases and Birth Defects of the Ministry of Education, Sichuan University, Chengdu, Sichuan, China; Key Laboratory of Development and Maternal and Child Diseases of Sichuan Province, China.
Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu 610041, China; Key Laboratory of Obstetrics & Gynecologic and Pediatric Diseases and Birth Defects of the Ministry of Education, Sichuan University, Chengdu, Sichuan, China; Key Laboratory of Development and Maternal and Child Diseases of Sichuan Province, China.
Clin Neurol Neurosurg. 2023 Oct;233:107955. doi: 10.1016/j.clineuro.2023.107955. Epub 2023 Aug 29.
To describe the clinical characteristics of anti-NMDAR encephalitis secondary to acute necrotizing encephalopathy caused by herpes simplex virus encephalitis in infants, and aid in its early recognition, diagnosis and treatment.
A total of 4 infants were included; all presented with fever, seizures, and progressive disturbances of consciousness and were diagnosed with herpes simplex virus (HSV-1) encephalitis. Cerebrospinal fluid (CSF) protein levels progressively increased, and the head MRI showed necrotizing encephalopathy. There was no significant improvement or recurrence after treatment with acyclovir, dexamethasone, or immunoglobulins. CSF reexamination at 3 weeks to 3 months showed positive anti-NMDAR IgG antibodies and gradual improvement after high-dose methylprednisolone therapy.
Infants with ANE associated with HSV can develop secondary anti-NMDAR encephalitis, recognition of which is critical to ensure the appropriate institution of immunotherapy after active CNS infection has been ruled out.
描述抗 NMDAR 脑炎继发于单纯疱疹病毒脑炎引起的婴儿急性坏死性脑病的临床特征,以帮助早期识别、诊断和治疗。
共纳入 4 例婴儿,均表现为发热、抽搐和进行性意识障碍,被诊断为单纯疱疹病毒(HSV-1)脑炎。脑脊液(CSF)蛋白水平逐渐升高,头部 MRI 显示坏死性脑病。阿昔洛韦、地塞米松或免疫球蛋白治疗后无明显改善或复发。CSF 复查在 3 周到 3 个月时显示抗 NMDAR IgG 抗体阳性,大剂量甲基强的松龙治疗后逐渐改善。
与 HSV 相关的ANE 可继发抗 NMDAR 脑炎,认识到这一点对于在排除中枢神经系统感染后的主动免疫治疗非常重要。