TY - JOUR
T1 - Multifocal fibrosclerosis with hypertrophic intracranial pachymeningitis
AU - Berger, J. R.
AU - Snodgrass, S.
AU - Glaser, J.
AU - Post, M.
AU - Norenberg, M.
AU - Benedetto, P.
PY - 1989/10
Y1 - 1989/10
N2 - 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.
AB - 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.
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U2 - 10.1212/wnl.39.10.1345
DO - 10.1212/wnl.39.10.1345
M3 - Article
C2 - 2797457
AN - SCOPUS:0024418060
VL - 39
SP - 1345
EP - 1349
JO - Neurology
JF - Neurology
SN - 0028-3878
IS - 10
ER -