Fulminant Idiopathic Intracranial Hypertension

Abstract

Fulminant idiopathic intracranial hypertension is extremely rare but invariably is associatedwith permanent blindness unless there is prompt intervention. Hypothetically, a rapid increase in pressure in the perineural space around the optic nerve can lead to ischemic opticneuropathyandblindness.1An 18-year-oldoverweightwoman with rapidly progressive visual loss over 4 days (leading to no light perception), nonreactive pupils, binocular abduction deficit suggestiveof sixthnervepalsies,bilateraloptic-discedema(Frisenstage 5),2 and cerebrospinal fluid opening pressure at 600 mm H2O receivedadiagnosisof fulminant idiopathic intracranialhypertension.1 The results of cerebrospinal fluid laboratory studies,magnetic resonance imaging of the brain, and vascular imagingwere normal. Our Figure, AandB, showsmagnetic resonance imagingscansof thepatient’s orbits at the time of presentation. Anemergent,controlled lumbardrainwasput inplace,andtreatment with intravenous methylprednisolone sodium succinate and furosemide was started on the first day. Five days later, her visual acuitywas20/400 inbotheyes, herpapilledema improved, andher abduction deficits resolved. Our Figure, C and D, shows follow-up magnetic resonance imaging scans on day 5. A ventriculoperitoneal shunt was performed for relatively long-term control of cerebrospinal fluidpressure.Onday9, herpapilledema improved toFri-