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Idiopathic hypertrophic cranial pachymeningitis
S R Hamilton1, C H Smith, S Lessell
1Division of Neurology, University of Washington, Seattle.
Journal of Clinical Neuro-Ophthalmology
|June 1, 1993
Summary
Hypertrophic cranial pachymeningitis is a rare condition causing chronic headaches and cranial nerve issues. Characteristic MRI findings of thickened, enhancing dura can aid in diagnosis.
Area of Science:
- Neurology
- Radiology
- Pathology
Background:
- Hypertrophic cranial pachymeningitis (HCP) is an uncommon condition characterized by thickening of the dura mater.
- Idiopathic HCP, unrelated to systemic diseases, presents diagnostic challenges.
Observation:
- Three patients with biopsy-proven HCP, unrelated to systemic disease, were evaluated.
- Clinical presentation included chronic headache, cranial neuropathy (bilateral sixth nerve palsies or optic neuropathies), elevated erythrocyte sedimentation rate (ESR), and mild cerebrospinal fluid (CSF) pleocytosis.
Findings:
- Magnetic resonance (MR) imaging demonstrated thickened dura mater with enhancement after gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA) administration.
- Histopathological examination revealed thickened, fibrotic dura with sterile, chronic, nongranulomatous inflammation.
- Treatment response to corticosteroids, immunosuppressive drugs, or radiation was variable.
Implications:
- The distinctive MR imaging appearance of thickened, enhancing dura is crucial for recognizing HCP.
- Early diagnosis and appropriate management can potentially improve patient outcomes in this rare neurological disorder.