Marked inflammation in catastrophic longitudinal myelitis associated with systemic lupus erythematosus

Catastrophic longitudinal myelitis is an extremely rare neurologic manifestation of collagen vascular disease, described heretofore in 11 cases of SLE and 1 of Sjogren's Syndrome. This report documents markedly abnormal and worsening CSF findings on sequential CSF examinations over a period of three days (WBC > 1500 cells/μL, >80% neutrophils, markedly elevated protein, and extremely low glucose levels) in the absence of infection. These abnormalities cleared rapidly with institution of immunosuppressive therapy so that a third CSF exam done within three days revealed almost complete normalization of CSF values. These findings suggest that in some cases of CLM a strong inflammatory component may be present, while in others, other pathogenic factors may predominate.