Andrews J M, Thompson J A, Pysher T J, Walker M L, Hammond M E
Department of Pathology, University of Utah School of Medicine, Salt Lake City.
Ann Neurol. 1990 Jul;28(1):88-90. doi: 10.1002/ana.410280116.
Refractory epilepsy, electroencephalographic abnormalities, progressive hemiplegia, and contralateral hemicerebral atrophy developed in a previously healthy 3-year, 9-month-old girl. Extensive laboratory testing showed elevated serum antinuclear antibody titers, cerebrospinal fluid oligoclonal bands, and elevated immunoglobulin G (IgG): albumin ratio, IgG index, and IgG synthesis rate. Pathological study of a subtotal hemispherectomy specimen revealed widespread cerebral vasculitis with immunofluorescence staining for IgG, IgM, IgA, C3, and Clq, and ultrastructural evidence of vascular injury in addition to severe cortical atrophy with marked neuronal loss. Cerebrospinal fluid abnormalities in other reported patients suggest that immunological abnormalities may not be unique to this girl. These data suggest possible immunopathogenetic mechanisms in these patients.
一名既往健康的3岁9个月女童出现难治性癫痫、脑电图异常、进行性偏瘫和对侧大脑半球萎缩。广泛的实验室检查显示血清抗核抗体滴度升高、脑脊液寡克隆带以及免疫球蛋白G（IgG）：白蛋白比值、IgG指数和IgG合成率升高。大脑半球次全切除标本的病理研究显示广泛的脑血管炎，IgG、IgM、IgA、C3和Clq免疫荧光染色阳性，除严重皮质萎缩伴明显神经元丢失外，还有血管损伤的超微结构证据。其他报道患者的脑脊液异常表明免疫异常可能并非该女童所特有。这些数据提示了这些患者可能的免疫发病机制。
