Goenka Ajay, Jain Vivek, Nariai Hiroki, Spiro Alfred, Steinschneider Mitchell
Saul R. Korey Department of Neurology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York.
Santokba Durlabhji Memorial Hospital, Jaipur, Rajasthan, India.
Pediatr Neurol. 2017 Jul;72:51-55. doi: 10.1016/j.pediatrneurol.2017.03.010. Epub 2017 Apr 5.
There is a wide spectrum of clinical manifestations in children with anti-N-methyl-d-aspartate (NMDA) receptor antibody encephalitis from two different health care settings.
We describe our experience with 13 patients (median age, 7 years; range, 5 months to 19 years) presenting to tertiary referral centers in India and the United States.
Initial manifestations were neurological (seizures or movement disorders) in eight patients, and psychiatric (e.g., emotional lability and hallucination) in five patients. Symptoms during the clinical course included seizures in ten patients, movement disorders (dyskinesia and choreiform movements) in 11 patients, and behavioral changes (aggressiveness and insomnia) in ten patients. Concomitant infections (herpes simplex virus 1, tuberculous meningitis, and influenza A) were present in three patients. Analysis of the cerebrospinal fluid in all except two cases preceded by infection (herpes simplex virus encephalitis and tuberculous meningitis) was unremarkable. Treatment included intravenous immunoglobulin/methylprednisolone (11 patients), rituximab (eight patients), plasmapheresis (two patients), and cyclophosphamide (two patients). Six patients recovered completely. Two patients had mild residual neurological deficits, whereas four had severe residual neurological deficits. Two patients had profound autonomic instability, which was the cause of death for one of them. Two patients relapsed at two and six months after the initial recovery.
We describe the differences and similarities of clinical presentation, test results, and response to treatment of children with anti-N-methyl-d-aspartate receptor encephalitis from India and the United States. Included is a description of one of the youngest patients with anti-N-methyl-d-aspartate receptor encephalitis (five months) and the first patient to be reported in association with tuberculous meningitis.
来自两种不同医疗环境的抗N-甲基-D-天冬氨酸（NMDA）受体抗体脑炎患儿存在广泛的临床表现。
我们描述了13例（中位年龄7岁；范围5个月至19岁）就诊于印度和美国三级转诊中心的患者的情况。
8例患者的初始表现为神经症状（癫痫发作或运动障碍），5例患者为精神症状（如情绪不稳定和幻觉）。临床病程中的症状包括10例患者癫痫发作、11例患者运动障碍（运动障碍和舞蹈样动作）以及10例患者行为改变（攻击性和失眠）。3例患者合并感染（单纯疱疹病毒1型、结核性脑膜炎和甲型流感）。除2例在感染（单纯疱疹病毒性脑炎和结核性脑膜炎）之前的病例外，所有病例的脑脊液分析均无异常。治疗包括静脉注射免疫球蛋白/甲基强的松龙（11例患者）、利妥昔单抗（8例患者）、血浆置换（2例患者）和环磷酰胺（2例患者）。6例患者完全康复。2例患者有轻度神经功能缺损残留，而4例有严重神经功能缺损残留。2例患者有严重的自主神经功能不稳定，其中1例因此死亡。2例患者在初次康复后2个月和6个月复发。
我们描述了来自印度和美国的抗N-甲基-D-天冬氨酸受体脑炎患儿在临床表现、检查结果和治疗反应方面的异同。其中包括对抗N-甲基-D-天冬氨酸受体脑炎最年幼患者（5个月）之一以及首例与结核性脑膜炎相关报道患者的描述。
