Fulminant idiopathic intracranial hypertension

Abstract

Severe and rapidly progressive visual loss suggests "fulminant idiopathic intracranial hypertension" and should prompt aggressive management. Urgent surgery may be required in these patients, and temporizing measures such as repeat lumbar punctures, lumbar drainage, and IV steroids considered.

Unilateral Transverse Sinus Stenting of Patients with Idiopathic Intracranial Hypertension

“…5,6 Up to 3% of patients develop a fulminant or malignant course with rapidly progressive visual loss. [7][8][9] Treatments focus on normalization of intracranial pressure. Initial therapy usually consists of acetazolamide in conjunction with dietary counseling for weight loss.…”

A 24-year-old woman with rapidly progressing vision loss

“…1,2 Fulminant IIH is a particularly acute and rapidly progressive form of IIH, with an incidence of 2.2% to 2.9% of all new IIH cases. 3 Normally, vision loss in IIH is secondary to chronic papilledema 4 ; however, progressive vision loss is rare and may indicate secondary causes of increased ICP, such as a meningeal process or venous sinus thrombosis. 5,6 Fulminant IIH is an acutely severe and rapidly progressive form of IIH, with resultant permanent visual sequelae.…”

“…5,6 Fulminant IIH is an acutely severe and rapidly progressive form of IIH, with resultant permanent visual sequelae. 3,[7][8][9][10][11][12][13] It is defined as acute onset of signs and symptoms of intracranial hypertension; (<4 weeks between onset of initial symptoms and severe visual loss), rapid worsening of vision loss over several days, and a normal MRI and MRV (or CT venogram). 3 There is limited literature on fulminant IIH.…”

Fulminant Idiopathic Intracranial Hypertension With Malignant Systemic Hypertension—A Case Report

“…In fulminant IIH-defined as (i) the acute onset of symptoms and signs of intracranial hypertension (less than 4 weeks between onset of initial symptoms and severe visual loss) and (ii) rapid worsening of visual loss over few days-there are reports of up to one quarter with coexistent systemic hypertension at presentation. 2 Fulminant IIH is a medical emergency and should be recognised early and managed as such. The coexistence of systemic hypertension with IIH has implications in terms of treatment and may indicate a group of patients at more risk of visual complications.…”