Viliuisk encephalomyelitis: intrathecal synthesis of oligoclonal IgG.
Viliuisk encephalomyelitis (VE) is a neurodegenerative disorder expressed as subacute meningo-encephalitis progressing to a more prolonged pan-encephalitic syndrome with a fatal outcome within 1 to 10 years. Some patients survive to a steady state of global dementia and severe spasticity that may last for over 20 years. Multiple micronecrotic foci surrounded by inflammatory infiltrates are observed throughout the cerebral cortex and other gray matter areas. Infectious etiology of VE is strongly suspected, but the causative agent has not been identified. We conducted a search for assays that might be helpful for VE diagnosis and established for the first time that the majority of patients with definite VE show evidence for intrathecal IgG synthesis correlating with the clinical manifestations of the disease. This indicates that the detection of oligoclonal IgG banding in the cerebrospinal fluid is a valuable diagnostic assay for VE. Implications of these findings for a possible etiology of VE are discussed.
Green
Alison J E
AJ
The National CJD Surveillance Unit, Western General Hospital, Edinburgh EH4 2XU, UK.
Sivtseva
Tatiana M
TM
Danilova
Al'bina P
AP
Osakovsky
Vladimir L
VL
Vladimirtsev
Vsevolod A
VA
Zeidler
Martin
M
Knight
Richard S
RS
Platonov
Feodor A
FA
Shatunov
Alexey
A
Alekseev
Vasiliy P
VP
Krivoshapkin
Vadim G
VG
Masters
Colin L
CL
Gajdusek
D Carleton
DC
Goldfarb
Lev G
LG
D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D015153:Blotting, Western / Q000379:methods; D002540:Cerebral Cortex / Q000473:pathology; D004679:Encephalomyelitis / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology*; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000276:immunology / Q000378:metabolism*; D007525:Isoelectric Focusing / Q000379:methods; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D043025:Oligoclonal Bands; D012016:Reference Values; D012680:Sensitivity and Specificity
D007136:Immunoglobulins; D043025:Oligoclonal Bands