Orbital apex syndrome associated with diffuse hypertrophic pachymeningitis in isolated neurosarcoidosis.

A 43-year-old woman reported acute onset of right retro-orbital headache followed by ptosis, paralysis of extraocular muscles, and subsequently amaurosis, characterizing an orbital apex syndrome. Brain MRI showed diffuse pachymeningitis with leptomeningeal and parenchymatous involvement (). CSF analysis was unremarkable. ACE and IgG4 levels were normal. However, investigation was done under oral steroid use. Lymphoproliferative and exocrine gland disorders were excluded. Lung scintigraphy and CT were normal. Meningeal biopsy disclosed diffuse non-caseous granulomatosis. Intravenous steroid pulse improved ocular movements (), [...]