Multifocal fibrosclerosis with hypertrophic intracranial pachymeningitis

Abstract

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.