Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, Tokyo, Japan.
Acta Neurol Scand. 2010 Apr;121(4):251-6. doi: 10.1111/j.1600-0404.2009.01198.x. Epub 2009 Dec 17.
We conducted a nationwide multicenter study in Japan to elucidate the clinical and laboratory characteristics of acute encephalitis with refractory, repetitive partial seizures (AERRPS).
Clinical and laboratory features, treatment, and outcome were assessed using a structured questionnaire.
Twenty-nine children were enrolled in the study. Refractory and repetitive partial seizures accompanied by fever were the cardinal clinical features. Partial seizures consisted principally of eye deviation or facial twitching, being periodically repeated during the acute phase. These seizures were refractory to conventional anticonvulsants and were only suppressed by high-dose intravenous barbiturate administration. Rhythmic activities on electroencephalography and non-specific cerebral atrophy on neuroimaging were common. Serum or cerebrospinal antibodies against GluRepsilon2 were positive in six patients. General prognosis was unfavorable due to intractable epilepsy and cognitive deficits.
Based on the peculiar and homogenous features, AERRPS can be regarded as a distinct clinical entity.
我们在日本进行了一项全国多中心研究，旨在阐明伴有难治性、复发性部分性癫痫发作的急性脑炎（AERRPS）的临床和实验室特征。
使用结构化问卷评估临床和实验室特征、治疗和结局。
研究纳入 29 名儿童。难治性和复发性伴发热的部分性癫痫发作是主要的临床特征。部分性发作主要表现为眼球偏斜或面部抽搐，在急性期周期性重复。这些癫痫发作对常规抗癫痫药物耐药，仅通过大剂量静脉注射巴比妥类药物才能抑制。脑电图上的节律性活动和神经影像学上的非特异性脑萎缩很常见。6 名患者的血清或脑脊液中存在针对 GluRepsilon2 的抗体呈阳性。由于难治性癫痫和认知障碍，总体预后不佳。
基于其独特且同质的特征，AERRPS 可视为一种明确的临床实体。
