Multifocal fibrosclerosis with hypertrophic intracranial pachymeningitis

J. R. Berger, S. Snodgrass, J. Glaser, M. Post, M. Norenberg, P. Benedetto

Research output: Contribution to journalArticle

85 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1345-1349
Number of pages5
JournalNeurology
Volume39
Issue number10
StatePublished - Oct 1989

ASJC Scopus subject areas

  • Clinical Neurology

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  • Cite this

    Berger, J. R., Snodgrass, S., Glaser, J., Post, M., Norenberg, M., & Benedetto, P. (1989). Multifocal fibrosclerosis with hypertrophic intracranial pachymeningitis. Neurology, 39(10), 1345-1349.