Idiopathic hypertrophic cranial pachymeningitis

Report of three cases

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✓ Hypertrophic cranial pachymeningitis is a rare, idiopathic form of granulomatous pachymeningitis. This report describes three cases of hypertrophic cranial pachymeningitis and discusses the clinical, radiographic, and pathological findings in these and other reported cases. These lesions typically cause progressive cranial nerve palsies, headaches, and cerebellar dysfunction. They occur in patients of all age groups; the peak incidence is in the sixth decade. Hypertrophic cranial pachymeningitis is best identified by magnetic resonance imaging. The diagnosis is established by excluding all other granulomatous and infectious diseases. A dural biopsy is essential to confirm the diagnosis. Hypertrophic cranial pachymeningitis is initially responsive to steroid therapy, but in most cases it recurs or progresses despite treatment. Surgical excision of granulomas is occasionally necessary to alleviate a mass effect. The long-term outcome remains uncertain for most patients, but progressive disease is usually fatal owing to cranial neuropathies.

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Address reprint requests to: Mark L. Rosenblum, M.D., Department of Neurological Surgery, c/o The Editorial Office, 1360 Ninth Avenue, Suite 210, San Francisco, California 94122.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 1. A and B: Magnetic resonance (MR) images showing a dural-based lesion at the level of the medulla causing brain-stem compression. The lesion is hyperintense on the T1-weighted image (A) and hypointense on the T2-weighted image (B). C: Coronal T1-weighted MR image showing diffuse dural thickening, especially at the levels of the tentorium and falx. D: Photomicrograph of a frontal dura biopsy specimen showing chronic inflammation, giant cells, and fibrosis, as well as some areas of acute inflammation. H & E. × 25.

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    Case 2. Upper Pair: Magnetic resonance images of the clivus region showing a ventrally based dural lesion that compresses the brain stem anteriorly. Lower: Photomicrograph of this lesion showing chronic and acute inflammatory changes with caseous necrosis, well-formed granulomata, giant cells, and fibrosis. H & E. × 48.

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    Case 3. Left: Computerized tomography scan showing diffuse dural thickening over the entire cerebrum with enhancement of the cavernous sinuses. Right: Photomicrograph of the dura of the cavernous sinus showing chronic inflammatory changes, necrosis, and granuloma formation. On gross examination, the dura was reddened, irregular, and nodular. H & E. × 50.

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