Analysis of the systemic and intrathecal humoral immune response in progressive multifocal leukoencephalopathy.
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.
Weber
T
T
Neurologische Klinik, Marienkrankenhaus Hamburg, Germany.
Trebst
C
C
Frye
S
S
Cinque
P
P
Vago
L
L
Sindic
C J
CJ
Schulz-Schaeffer
W J
WJ
Kretzschmar
H A
HA
Enzensberger
W
W
Hunsmann
G
G
Lüke
W
W
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*
D000914:Antibodies, Viral; D036022:Capsid Proteins; D007074:Immunoglobulin G; C062481:VP1 protein, polyomavirus