Adams C, Diadori P, Schoenroth L, Fritzler M
Alberta Children's Hospital and Faculty of Medicine, University of Calgary, Canada.
Can J Neurol Sci. 2000 Nov;27(4):316-20. doi: 10.1017/s0317167100001074.
Anti-Purkinje cell antibodies have been reported in cerebellar ataxia following Epstein-Barr virus (EBV) infection. We investigated autoantibody responses, including anti-Purkinje cell antibodies, and the clinical course in eight children who developed post-varicella ataxia, five of their siblings with uncomplicated varicella, one child with post-EBV ataxia, two children with acute disseminated encephalomyelitis (ADEM) and one with neuroblastoma associated ataxia, and in age and gender matched controls.
Autoantibodies were tested by indirect immunofluorescence (IIF) on cryopreserved cerebrum and cerebellum sections. Other autoantibodies were measured by conventional IIF protocols using HEp-2 cells as a substrate. Antibodies to myelin associated glycoprotein (MAG), asialo-GM1, beta2 glycoprotein 1, cardiolipin and myelin basic protein (MBP) were measured by ELISA.
Three of eight children with acute post-varicella ataxia, one child with post-EBV ataxia, one child with ADEM and one child with uncomplicated varicella, had high titer autoantibodies (>1/160) that reacted with cerebrum and cerebellar tissue. This reactivity was not seen in one child with ADEM, in one with neuroblastoma and ataxia, in the remainder of the children with uncomplicated varicella or age and gender matched controls. Autoantibodies were not seen in CSF from two children with post-varicella ataxia. The punctate staining seen on cerebrum and cerebellum sections co-localized with rabbit antibodies to the centrosome protein pericentrin. All patients with strong reactivity with cerebrum and cerebellar tissue by IIF had elevated levels of anti-MAG that was not confirmed by absorption assay. No reactivity was seen with asialo-GM1, MBP, beta2 glycoprotein 1 or cardiolipin. None of the sera had autoantibodies directed against endosomes, the Golgi complex, or the paraneoplastic autoantigens Hu and Yo.
Some children with post-viral ataxia develop antibodies that have strong reactivity with cerebral and cerebellar tissue. Some of the antigenic reactivity co-localized with the centrosome protein pericentrin.
据报道，在感染爱泼斯坦-巴尔病毒（EBV）后的小脑共济失调患者中可检测到抗浦肯野细胞抗体。我们调查了8例患水痘后共济失调的儿童、5例患单纯性水痘的同胞、1例患EBV感染后共济失调的儿童、2例患急性播散性脑脊髓炎（ADEM）的儿童和1例患神经母细胞瘤相关性共济失调的儿童以及年龄和性别匹配的对照组的自身抗体反应，包括抗浦肯野细胞抗体，以及临床病程。
采用间接免疫荧光法（IIF）检测冻存的大脑和小脑切片上的自身抗体。其他自身抗体采用以人喉癌上皮细胞（HEp-2）为底物的传统IIF方法检测。采用酶联免疫吸附测定法（ELISA）检测抗髓鞘相关糖蛋白（MAG）、去唾液酸GM1、β2糖蛋白1、心磷脂和髓鞘碱性蛋白（MBP）的抗体。
8例急性水痘后共济失调患儿中的3例、1例EBV感染后共济失调患儿、1例ADEM患儿和1例单纯性水痘患儿，其自身抗体滴度较高（>1/160），与大脑和小脑组织发生反应。1例ADEM患儿、1例神经母细胞瘤伴共济失调患儿、其余患单纯性水痘的患儿以及年龄和性别匹配的对照组中均未出现这种反应性。2例水痘后共济失调患儿的脑脊液中未检测到自身抗体。大脑和小脑切片上出现的点状染色与兔抗中心体蛋白中心粒外周蛋白抗体共定位。所有通过IIF与大脑和小脑组织发生强烈反应的患者，其抗MAG水平均升高，但吸收试验未证实这一点。未观察到与去唾液酸GM1、MBP、β2糖蛋白1或心磷脂的反应性。所有血清中均未检测到针对内体、高尔基体或副肿瘤自身抗原Hu和Yo的自身抗体。
一些病毒感染后共济失调患儿会产生与大脑和小脑组织发生强烈反应的抗体。部分抗原反应性与中心体蛋白中心粒外周蛋白共定位。
