Lyon G, Griscelli C, Fernandez-Alvarez E, Prats-Vinas J, Lebon P
Neuropadiatrie. 1980 Feb;11(1):57-71. doi: 10.1055/s-2008-1071375.
This report is on six cases of a chronic relentlessly progressive encephalitis occurring in boys with congenital hypogammaglobulinemia presumably of the x-linked type, which are thought to represent a separate neurological entity. Intellectual deterioration, dysarthria, spasticity, ataxia, optic atrophy and an increase of lymphocytes in the cerebrospinal fluid, were the main clinical signs. The pathological picture was that of a viral encephalitis, but all virological investigations on brain biopsies and CSF were negative. The significance of intra-cisternal tubuloreticular inclusions in brain endothelial cells, similarities with chronic rubella encephalitis, and the role of the immunological deficiency are discussed. Sofar, the cause of this new type of encephalitis remains obscure.
本报告涉及6例患有先天性低丙种球蛋白血症（推测为X连锁型）的男孩所发生的慢性进行性脑炎，认为这代表一种独立的神经疾病实体。智力衰退、构音障碍、痉挛状态、共济失调、视神经萎缩以及脑脊液中淋巴细胞增多，是主要的临床体征。病理表现为病毒性脑炎，但对脑活检和脑脊液进行的所有病毒学检查均为阴性。文中讨论了脑内皮细胞内池内小管状网状包涵体的意义、与慢性风疹脑炎的相似之处以及免疫缺陷的作用。迄今为止，这种新型脑炎的病因仍不清楚。
