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Multifocal fibrosclerosis with hypertrophic intracranial pachymeningitis

Department of Neurology, University of Miami School of Medicine, Miami, FL. (Drs. Berger and Snodgrass)
Internal Medicine, University of Miami School of Medicine, Miami, FL. (Dr. Berger)
Ophthalmology, University of Miami School of Medicine, Miami, FL. (Dr. Glaser)
Radiology, University of Miami School of Medicine, Miami, FL. (Dr. Post)
Pathology, University of Miami School of Medicine, Miami, FL. (Dr. Norenberg)
Oncology, University of Miami School of Medicine, Miami, FL. (Dr. Benedetto), University of Miami School of Medicine, Miami, FL.

We describe a 34-year-old man with a 17-year history of multifocal fibrosclerosis resulting in unique neurologic complications. Episcleritis, orbital pseudotumor, and sclerosing cholangitis accompanied a florid intracranial inflammatory pachymeningitis. The latter was associated with blindness, multiple cranial neuropathies, pseudotumor cerebri, and seizures. Extensive investigation failed to reveal an etiology. Corticosteroids were ineffective in preventing progression of the disorder, and the value of antineoplastic therapy was uncertain. Multifocal fibrosclerosis, a rare disorder, may result in a confusing array of neurologic manifestations.

Address correspondence and reprint requests to Dr. Berger, Department of Neurology, University of Miami School of Medicine, 1501 N.W. 9th Avenue, Miami, FL 33136.

Received March 30, 1989. Accepted for publication in final form April 28, 1989.

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