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Hypertrophic pachymeningitis as an initial and cardinal manifestation of microscopic polyangiitis

Yutaka Furukawa1, Yasuko Matsumoto, Masahito Yamada

  • 1Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, 13-1, Takara-machi, Kanazawa, Ishikawa 920-8640, Japan. furukawa@med.kanazawa-u.ac.jp

Neurology
|November 10, 2004
PubMed

Insights

Microscopic polyangiitis can initially manifest as hypertrophic pachymeningitis. This rare presentation involves inflammation of the dura mater and is associated with antineutrophil cytoplasmic antibodies.

Area of Science:

  • Neurology
  • Nephrology
  • Rheumatology

Background:

  • Microscopic polyangiitis (MPA) is a systemic vasculitis.
  • Hypertrophic pachymeningitis is thickening of the dura mater.
  • MPA typically affects small blood vessels.

Observation:

  • A 68-year-old man presented with hypertrophic pachymeningitis.
  • The patient had high antineutrophil cytoplasmic antibody (ANCA) titers for myeloperoxidase.
  • This was the initial and primary symptom of his condition.

Findings:

  • Kidney biopsy showed necrotizing glomerulonephritis.
  • Sural nerve biopsy revealed small vessel vasculitis.
  • Dura mater infiltration included plasma cells and eosinophils.

Implications:

  • Hypertrophic pachymeningitis can be an early sign of microscopic polyangiitis.
  • This case highlights the diverse neurological manifestations of MPA.
  • Early recognition is crucial for timely diagnosis and treatment of MPA.

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