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Scoring Central Nervous System Inflammation, Demyelination, and Axon Injury in Experimental Autoimmune Encephalomyelitis
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Marked inflammation in catastrophic longitudinal myelitis associated with systemic lupus erythematosus

A C Heinlein1, E Gertner

  • 1Section of Rheumatology, Regions Hospital and University of Minnesota Medical School, MN, USA.

Lupus
|September 27, 2007
PubMed

Insights

Catastrophic longitudinal myelitis (CLM), a rare neurological condition, shows significant cerebrospinal fluid (CSF) abnormalities. Prompt immunosuppressive therapy rapidly normalized these inflammatory CSF markers, suggesting CLM

Area of Science:

  • Neurology
  • Immunology
  • Rheumatology

Background:

  • Catastrophic longitudinal myelitis (CLM) is an exceptionally rare neurological complication of collagen vascular diseases.
  • Previous reports link CLM to Systemic Lupus Erythematosus (SLE) and Sjogren's Syndrome.

Observation:

  • This case report details sequential cerebrospinal fluid (CSF) findings over three days in a patient with CLM.
  • CSF analysis revealed severe abnormalities: high white blood cell count (>1500 cells/microL) with neutrophil predominance (>80%), markedly elevated protein, and critically low glucose levels.
  • These CSF abnormalities were observed in the absence of any detectable infection.

Findings:

  • Rapid and significant improvement in CSF parameters was noted within three days of initiating immunosuppressive therapy.
  • CSF examination demonstrated near-complete normalization of previously abnormal values.
  • The rapid resolution of CSF abnormalities suggests a substantial inflammatory component in this case of CLM.

Implications:

  • These findings highlight the potential role of inflammation in the pathogenesis of CLM.
  • The results suggest that prompt immunosuppressive treatment may be effective in managing CLM.
  • Further research is warranted to elucidate the diverse pathogenic mechanisms underlying CLM.

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