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 greater than or equal to 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.
Weber, T., Trebst, C., Frye, S., Cinque, P., Vago, L., Sindic, C., Schulz-Schaeffer, W. J., Kretzschmar, H. A., Enzensberger, W., Hunsmann, G., & Lüke, W. (1997). Analysis of the systemic and intrathecal humoral immune response in progressive multifocal leukoencephalopathy. The Journal of Infectious Diseases, 176(1), 250-254. https://doi.org/10.1086/514032 (Original work published 1997)