Bartram C R, Henke J, Treuner J, Basler M, Esch A, Mortier W
Eur J Pediatr. 1982 Mar;138(2):187-90. doi: 10.1007/BF00441152.
This paper describes the very rare occurrence of subacute sclerosing panencephalitis (SSPE) in two siblings: a Turkish boy and his younger sister. The clinical picture was characteristic, and the diagnosis was confirmed in both cases by appropriate laboratory examination. The interval between the occurrence of the first neurological symptoms in the boy, and subsequently in the girl was four years. Study of HLA- and 27 other polymorphic marker-systems did not reveal linkage to one of the systems tested. Therapeutic trials in the girl included intravenous and intraventricular application of a total of 87 X 10(6) U human fibroblast interferon (Hu INF-beta) over 21 days. However, up to 3 months after the end of interferon administration there were no significant changes in the girl's condition.
本文描述了在一对姐弟(一名土耳其男孩及其妹妹)中极为罕见的亚急性硬化性全脑炎(SSPE)病例。临床表现具有特征性,两例均通过适当的实验室检查得以确诊。男孩出现首例神经症状与随后女孩出现神经症状之间的间隔为四年。对HLA及其他27个多态性标记系统的研究未发现与所检测的任何一个系统存在连锁关系。对女孩的治疗试验包括在21天内静脉和脑室内共应用87×10⁶U人成纤维细胞干扰素(Hu INF-β)。然而,在干扰素给药结束后长达3个月的时间里,女孩的病情并无显著变化。