Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis
Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis
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DOI：
10.1111/j.1600-0404.2009.01198.x
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发表时间：
2010-04-01
影响因子：
3.5
通讯作者：
Sasaki, M.
中科院分区：
文献类型：
--
作者：
Sakuma, H.;Awaya, Y.;Sasaki, M.
目的-我们在日本进行了一项全国性的多中心研究，以阐明急性脑炎与难治性，重复性部分性癫痫发作（AERRPS）的临床和实验室特征。材料和方法-临床和实验室特征，治疗和结果进行了评估，使用结构式questionnaire.Results -29名儿童参加了这项研究。难治性和反复性部分性发作伴发热是主要临床特征。部分性癫痫发作主要表现为眼偏斜或面部抽搐，在急性期周期性重复。这些癫痫发作是难治性的常规抗惊厥药，只能抑制高剂量静脉注射巴比妥类药物。脑电图节律性活动和神经影像学非特异性脑萎缩是常见的。6例患者血清或脑脊液中抗GluR β 2抗体阳性。由于难治性癫痫和认知功能障碍，一般预后不良。结论-基于独特的和同质的功能，AERRPS可以被视为一个独特的临床实体。
Objective -We conducted a nationwide multicenter study in Japan to elucidate the clinical and laboratory characteristics of acute encephalitis with refractory, repetitive partial seizures (AERRPS).Materials and methods -Clinical and laboratory features, treatment, and outcome were assessed using a structured questionnaire.Results -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 GluR epsilon 2 were positive in six patients. General prognosis was unfavorable due to intractable epilepsy and cognitive deficits.Conclusion -Based on the peculiar and homogenous features, AERRPS can be regarded as a distinct clinical entity.