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Meningeal involvement in Wegener's granulomatosis

Ali Al Dhanhani1, Robert Macaulay, Bill Maloney

  • 1Division of Rheumatology, Department of Medicine, the Division of Anatomical Pathology, Queen Elizabeth II Health Sciences Centre and Dalhousie University, Halifax, Nova Scotia, Canada.

The Journal of Rheumatology
|February 9, 2006
PubMed

Insights

Wegener's granulomatosis (WG) can cause rare neurological symptoms due to meningeal involvement. Successful treatment involved cyclophosphamide and methotrexate, achieving long-term remission.

Area of Science:

  • Neurology
  • Rheumatology
  • Immunology

Background:

  • Wegener's granulomatosis (WG), a form of vasculitis, typically affects the respiratory tract and kidneys.
  • Neurological involvement in WG is uncommon, and diagnosis can be challenging.
  • Antineutrophil cytoplasmic antibodies (ANCA) are often used for diagnosis but can be negative in some WG cases.

Observation:

  • A patient presented with neurological symptoms suggestive of meningeal involvement.
  • Diagnosis of WG was complicated by persistently negative ANCA.
  • Histopathological findings from multiple sites lacked diagnostic specificity.

Findings:

  • The patient's neurological symptoms were attributed to meningeal involvement secondary to WG.
  • Treatment with oral cyclophosphamide led to successful remission of symptoms.
  • Long-term remission (3 years) was maintained with oral methotrexate.

Implications:

  • This case highlights a rare neurological manifestation of WG.
  • It underscores the importance of considering WG even with negative ANCA and non-specific pathology.
  • The successful treatment regimen suggests a potential therapeutic strategy for similar cases.

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