Idiopathic hypertrophic cranial pachymeningitis.
We evaluated 3 patients with biopsy-proven hypertrophic cranial pachymeningitis apparently unrelated to any systemic disease. Each patient had chronic headache, cranial neuropathy, an elevated ESR, and a mild CSF pleocytosis. Neuro-ophthalmic findings included bilateral sixth nerve palsies in two patients and the third had bilateral optic neuropathies. MR imaging revealed thickened dura that enhanced with Gd-DTPA administration. Histologic examination showed thickened, fibrotic dura with a sterile, chronic, nongranulomatous inflammation. The response to treatment was variable with corticosteroids, immunosuppressive drugs, or radiation. The distinctive MR appearance should help physicians recognize this rarely reported disease.
Hamilton
S R
SR
Division of Neurology, University of Washington, Seattle.
Smith
C H
CH
Lessell
S
S
D000010:Abducens Nerve / Q000473:pathology; D000328:Adult; D000368:Aged; D003287:Contrast Media; D003389:Cranial Nerve Diseases / Q000175:diagnosis; D004388:Dura Mater / Q000473:pathology*; D004660:Encephalitis / Q000175:diagnosis / Q000473:pathology*; D005260:Female; D005682:Gadolinium; D019786:Gadolinium DTPA; D006261:Headache / Q000175:diagnosis; D006801:Humans; D006984:Hypertrophy; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009901:Optic Nerve Diseases / Q000175:diagnosis; D009942:Organometallic Compounds; D010243:Paralysis / Q000175:diagnosis; D004369:Pentetic Acid
D003287:Contrast Media; D009942:Organometallic Compounds; D004369:Pentetic Acid; D005682:Gadolinium; D019786:Gadolinium DTPA