Viliuisk encephalomyelitis: intrathecal synthesis of oligoclonal IgG

Alison J E Green, Tatiana M Sivtseva, Al'bina P Danilova, Vladimir L Osakovsky, Vsevolod A Vladimirtsev, Martin Zeidler, Richard S Knight, Feodor A Platonov, Alexey Shatunov, Vasiliy P Alekseev, Vadim G Krivoshapkin, Colin L Masters, D Carleton Gajdusek, Lev G Goldfarb

Research output: Contribution to journalArticlepeer-review

10 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)69-73
Number of pages5
JournalJournal of the Neurological Sciences
Volume212
Issue number1-2
Publication statusPublished - 15 Aug 2003

Keywords

  • Adult
  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Blotting, Western
  • Cerebral Cortex
  • Encephalomyelitis
  • Female
  • Humans
  • Immunoglobulins
  • Isoelectric Focusing
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Oligoclonal Bands
  • Reference Values
  • Sensitivity and Specificity
  • Comparative Study
  • Journal Article

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