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Idiopathic hypertrophic pachymeningitis with anticardiolipin antibody: A case report

Chi-Shun Wu1, Hung-Ping Wang2, Sheng-Feng Sung1

  • 1Division of Neurology.

Medicine
|January 20, 2021
PubMed
Abstract

Insights

Idiopathic hypertrophic pachymeningitis (IHP) can be treated with methotrexate for steroid-resistant cases. Serial erythrocyte sedimentation rate (ESR) testing helps monitor treatment response in patients with this rare neurological disorder.

Area of Science:

  • Neurology
  • Pathology

Background:

  • Idiopathic hypertrophic pachymeningitis (IHP) is a rare neurological condition with an unknown cause.
  • Diagnosis relies on excluding other potential etiologies.

Observation:

  • A 41-year-old male presented with a 3-month history of headache.
  • MRI revealed diffuse pachymeningeal enhancement; lumbar puncture showed elevated pressure, lymphocytic pleocytosis, and protein.
  • Blood tests indicated elevated ESR and C-reactive protein.

Findings:

  • Pathology showed collagen deposition and lymphoid aggregation in the dura mater.
  • After excluding malignancy and infection, IHP was diagnosed.
  • Treatment with prednisolone, azathioprine, and methotrexate led to reduced headache severity and meningeal enhancement over 7 years.

Implications:

  • Methotrexate is a potential therapy for steroid-resistant IHP.
  • Serial ESR testing can guide treatment strategy and assess therapeutic response.

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