Characterisation and Visual Outcomes of Fulminant Idiopathic Intracranial Hypertension: A Narrative Review

ABSTRACT

Fulminant idiopathic intracranial hypertension (IIH) is a rapid vision-degrading presentation of IIH with limited published studies. This study composed a narrative review of fulminant IIH with the aim of better characterising fulminant IIH presentation and visual outcomes. SCOPUS and PubMed were searched for papers referencing IIH, benign intracranial hypertension, or pseudotumour cerebri. Abstracts were screened for rapid degradation in vision. All studies were required to meet both the modified Dandy and fulminant IIH criteria. Thirty-six studies met the inclusion criteria. Demographics, treatments, and visual outcome data were collected. Case studies made up 69% of the studies and 31% were case series. In total, 72 patients with fulminant IIH were reported, of which 23.6% were paediatric and 96% were female. Surgical intervention occurred in 85% of patients. Anaemia was present in 11% of patients and 85.7% of paediatric patients had a sixth cranial nerve palsy. In conclusion, we propose the following practice guidelines to assist in diagnosing and treating fulminant IIH patients: 1) patients who present with optic disc oedema require urgent visual field testing to evaluate for vision loss; 2) a paediatric patient presenting with a sixth cranial nerve palsy should have a comprehensive eye examination; 3) fulminant IIH can occur in patients with a normal body mass index; and 4) anaemia should be tested for in the setting of fulminant IIH. As little is known about the optimal treatment mechanisms for this presentation, multi-institutional and international collaborations will be a critical step for future research.

KEYWORDS:

• IIH
• optic neuropathy
• vision loss
• fulminant IIH
• pseudotumor cerebri

Disclosure statement

No potential conflict of interest was reported by the author(s).

Supplemental data

Supplemental data for this article can be accessed online at https://doi.org/10.1080/01658107.2023.2301358

Additional information

Funding

P30EY025585(BA-A), Research to Prevent Blindness (RPB) Challenge Grant, Cleveland Eye Bank Foundation Grant National Eye Institute: T32 EY024236 (Jacqueline Shaia) Rishi Singh reports personal fees from Genentech/Roche, Alcon, Novartis, Regeneron, Asclepix, Gyroscope, Bausch and Lomb, Apellis Katherine Talcott reports Alimera (consultant), Apellis (consultant), Bausch and Lomb (consultant), Eyepoint (consultant), Genentech (consultant, speaker’s bureau), Iveric Bio (speaker’s bureau), Regeneron (research), Regenxbio (research), Zeiss (research).