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NEUROLOGY 1993;43:1329
© 1993 American Academy of Neurology

Cranial pachymeningitis of unknown origin

A study of seven cases

C. Masson, MD, D. Hénin, MD, J. J. Hauw, MD, A. Rey, MD, P. Raverdy, MD and M. Masson, MD

Service de Neurologie (Drs. C. Masson and M. Masson), Laboratoire d'Anatomie et de Cytologie Pathologique (Dr. Hénin), Service de neurochirurgie (Dr. Rey), Hôpital Beaujon, Clichy; Laboratoire de Neuropathologie R. Escourolle (Dr. Hauw), Hôpital de La Salpêtrière, Paris; and the Service de Neurologie (Dr. Raverdy), Hôpital de Saint Germain, Saint Germain en Laye, France.

We report seven patients with cranial pachymeningitis of unknown origin in whom the main clinical features were headaches, ataxia, and cranial nerve palsies. CSF showed inflammatory changes. CT and MRI showed thickening of the falx and of the tentorium. The clinical course was chronic. Four patients improved with prednisolone but became steroid-dependent: in two cases, radiotherapy had no lasting improvement and in one, azathio-prine permitted a reduction of the corticosteroids. Five patients had biopsy of the tentorium cerebelli or of the temporal dura mater. In two cases, autopsy revealed extensive pachymeningitis without parenchymal changes. In all instances, microscopic examination of the dura mater showed a cellular infiltrate of polymorphic cells; there were no epithelioid granulomas. Review of the literature discloses seven similar cases. We discuss the relationship of these lesions with inflammatory meningeal masses, the focal pachymeningitis of the Tolosa-Hunt syndrome, and multifocal fibrosis.

Address correspondence and reprint requests to Dr. C. Masson, Hôpital Beaujon, 100 Bd du Général Leclerc, 92110 Clichy, France.

Received August 25, 1992. Accepted for publication in final form December 1, 1992.




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