We report three patients with chronic headaches and optic neuropathy d
ue to widespread meningeal thickening shown on enhanced MRI; all had b
iopsy-proven intracranial pachymeningitis (fibrosclerosis of the menin
ges). Two patients had bilateral optic neuropathy, elevated CSF protei
n, and polyclonal serum hypergammaglobulinemia. They developed tempora
l lobe cortical necrosis or sagittal sinus thrombosis, presumably due
to compromised dural venous drainage from extensive meningeal fibrosis
. The other patient had multiple cranial nerve palsies and unilateral
optic neuropathy with normal CSF. Corticosteroid therapy improved visu
al function in all three patients, although all had persisting visual
deficits. Gadolinium-enhanced MRI was essential in identifying meninge
al inflammation and locating suitable biopsy sites.