Tuxhorn Ingrid E B
Section of Pediatric Epilepsy, Epilepsy Center Bethel, Maraweg 21, 33617 Bielefeld, Germany.
Pediatr Neurol. 2004 Oct;31(4):291-4. doi: 10.1016/j.pediatrneurol.2004.03.015.
Subacute sclerosing panencephalitis is a form of chronic persistent measles encephalitis in childhood which rarely manifests after wild virus infection. One previous case of familial subacute sclerosing panencephalitis in two siblings and a number of reports of subacute sclerosing panencephalitis in one member of monozygous twins have been reported in the literature. This report describes a second sibling set who both contracted subacute sclerosing panencephalitis after concurrent sporadic measles infection. Two nonimmunized brothers developed neuropsychological decline and progressive myoclonic and complex partial seizures after earlier measles infection. Stereotyped periodic 5- to 8-second complexes in the electroencephalogram suggested the diagnosis of subacute sclerosing panencephalitis, subsequently confirmed by intrathecal and serum measles-specific immunoglobulin G synthesis and the presence of rubeola nucleocapsid protein and ribonucleic acid in the biopsied brain. The viral genome has not been further subtyped in either patient at this point. Although a rare event, subacute sclerosing panencephalitis cases involving familial and singular monozygous twins may shed light on a variety of host susceptibility factors and specific viral genotype features predisposing to this disease.
亚急性硬化性全脑炎是儿童期慢性持续性麻疹脑炎的一种形式，在野生病毒感染后很少出现。文献中曾报道过一例发生在两兄弟身上的家族性亚急性硬化性全脑炎病例，以及一些关于单卵双胞胎中一人患亚急性硬化性全脑炎的报道。本报告描述了另一对兄弟，他们在同时感染散发性麻疹后均患上了亚急性硬化性全脑炎。两名未接种疫苗的兄弟在早期感染麻疹后出现神经心理衰退、进行性肌阵挛和复杂部分性癫痫发作。脑电图中刻板的周期性5至8秒复合波提示亚急性硬化性全脑炎的诊断，随后经鞘内和血清麻疹特异性免疫球蛋白G合成以及活检脑组织中存在麻疹核衣壳蛋白和核糖核酸得以证实。目前这两名患者的病毒基因组均未进一步分型。尽管亚急性硬化性全脑炎病例涉及家族性和单卵双胞胎的情况罕见，但可能有助于揭示导致该疾病的多种宿主易感性因素和特定病毒基因型特征。
