Bilateral optic neuropathy associated with diffuse cerebral angiomatosis in Sturge-Weber syndrome.

Visual loss in patients with encephalotrigeminal angiomatosis or Sturge-Weber syndrome usually results from secondary glaucoma or from damage to the retrogeniculate pathways by the angiomatous lesions. Optic neuropathy has not been reported as a component of this syndrome. This report describes a patient who developed bilateral optic neuropathy with progressive visual loss associated with diffuse cerebral angiomatosis in the setting of Sturge-Weber syndrome.