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Granulomatosis with polyangiitis presenting with pachymeningitis

Nasarachi E Onyeuku1, Nikhil Balakrishnan2, Michael S Cartwright2

  • 1Wake Forest School of Medicine, United States.

Journal of the Neurological Sciences
|July 24, 2014
PubMed
Abstract

Insights

Granulomatosis with polyangiitis (GPA) can cause persistent headaches and cranial nerve palsies in older adults. Early diagnosis via MRI and ANCA testing is crucial for effective treatment.

Area of Science:

  • Neurology
  • Rheumatology
  • Immunology

Background:

  • Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis.
  • It commonly affects the respiratory tract and kidneys, but can involve the central nervous system.

Observation:

  • A 75-year-old male presented with daily headaches and multiple cranial nerve palsies.
  • Brain MRI revealed pachymeningitis, inflammation of the dura mater.

Findings:

  • Laboratory tests showed elevated proteinase-3 antineutrophil cytoplasmic antibodies (PR3-ANCA) and cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA).
  • These findings confirmed the diagnosis of GPA.

Implications:

  • GPA should be considered in the differential diagnosis of persistent headaches and pachymeningitis in elderly patients.
  • Contrast-enhanced brain MRI is a valuable tool for diagnosing CNS involvement in GPA.
Keywords:
Cranial neuropathyGranulomatosis with polyangiitisHeadacheMRIPachymeningitisWegener's granulomatosis

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