Idiopathic Hypertrophic Cranial Pachymeningitis

Diagnosis:

Rare, A diagnosis by exclusion
Note: Pachymeningitis is a radiologic pattern

Clinical features:

Severe headache, cranial nerve palsies, ataxia, loss of vision (optic nerve involvement)

Pathology:

Biopsy: Meningeal Polymorphonuclear infiltration

Findings on Investigations:

MRI:

  • T1 +contrast: enhancement of the meninges without enhancement of the sulci. Involves the tentorium, falx, calvarium
  • T2: hypointense dura

Investigations to consider:

LP, CSF analysis:

  • Opening pressure: low in spontaneous intracranial hypotension
  • Routine analysis: infectious meningitis
  • Cytology to rule out leptomeningeal carcinomatosis (carcinomatous meningitis)

Biopsy: sarcoidosis, plasmacytic tumors

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