进行性多灶性白质脑病的全身和鞘内体液免疫反应分析

Analysis of the systemic and intrathecal humoral immune response in progressive multifocal leukoencephalopathy.

作者信息

Weber T, Trebst C, Frye S, Cinque P, Vago L, Sindic C J, Schulz-Schaeffer W J, Kretzschmar H A, Enzensberger W, Hunsmann G, Lüke W

机构信息

Neurologische Klinik, Marienkrankenhaus Hamburg, Germany.

出版信息

J Infect Dis. 1997 Jul;176(1):250-4. doi: 10.1086/514032.

DOI:10.1086/514032

PMID:9207375

Abstract

Progressive multifocal leukoencephalopathy (PML) is a subacute viral infection of oligodendrocytes by JC virus occurring almost exclusively in immunocompromised patients. By use of partially purified recombinant VP1 as antigen, the IgG response was analyzed by a quantitative ELISA of paired cerebrospinal fluid (CSF) and serum samples. An intrathecal immune response to VP1, defined as an antibody-specificity index of CSF to serum antibody titers > or =1.5, was found in 76% of PML patients (47/62) but in only 3.2% of controls (5/155) (P < .001). Intra-blood-brain barrier synthesis of VP1-specific IgG antibodies is 76% sensitive and 96.8% specific for the diagnosis of PML. Furthermore, the excellent correlation (r = .985) between the plasma cell count in brain tissue and the humoral intrathecal immune response to VP1 in PML patients suggests a role for B cells in this disorder.

摘要

进行性多灶性白质脑病（PML）是由JC病毒引起的少突胶质细胞亚急性病毒感染，几乎仅发生于免疫功能低下的患者。以部分纯化的重组VP1作为抗原，通过对配对的脑脊液（CSF）和血清样本进行定量ELISA分析IgG反应。在76%的PML患者（47/62）中发现了针对VP1的鞘内免疫反应，定义为CSF与血清抗体滴度的抗体特异性指数≥1.5，但在仅3.2%的对照者（5/155）中发现（P<.001）。血脑屏障内VP1特异性IgG抗体的合成对PML诊断的敏感性为76%，特异性为96.8%。此外，PML患者脑组织中浆细胞计数与对VP1的体液鞘内免疫反应之间的良好相关性（r=.985）表明B细胞在这种疾病中起作用。