title: Analysis of the systemic and intrathecal humoral immune response in progressive multifocal leukoencephalopathy.
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.
authors.affiliation: Neurologische Klinik, Marienkrankenhaus Hamburg, Germany.
authors.forename: T
authors.initials: T
authors.lastname: Weber
authors.forename: C
authors.initials: C
authors.lastname: Trebst
authors.forename: S
authors.initials: S
authors.lastname: Frye
authors.forename: P
authors.initials: P
authors.lastname: Cinque
authors.forename: L
authors.initials: L
authors.lastname: Vago
authors.forename: C J
authors.initials: CJ
authors.lastname: Sindic
authors.forename: W J
authors.initials: WJ
authors.lastname: Schulz-Schaeffer
authors.forename: H A
authors.initials: HA
authors.lastname: Kretzschmar
authors.forename: W
authors.initials: W
authors.lastname: Enzensberger
authors.forename: G
authors.initials: G
authors.lastname: Hunsmann
authors.forename: W
authors.initials: W
authors.lastname: Lüke
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000914:Antibodies, Viral / Q000096:biosynthesis*; D002213:Capsid / Q000276:immunology*; D036022:Capsid Proteins; D002648:Child; D002675:Child, Preschool; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000096:biosynthesis; D007577:JC Virus / Q000276:immunology*; D007968:Leukoencephalopathy, Progressive Multifocal / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D013116:Spinal Cord / Q000276:immunology*
chemical_list: D000914:Antibodies, Viral; D036022:Capsid Proteins; D007074:Immunoglobulin G; C062481:VP1 protein, polyomavirus