Fulminant idiopathic intracranial hypertension (IIH) is a rapid vision-degrading presentation of IIH with limited published studies. This narrative review aims to collate current knowledge around fulminant IIH presentation and visual outcomes. Search terms included IIH, benign intracranial hypertension, or pseudo-tumour cerebri. Authors screened abstracts for rapid degradation in vision. Thirty-six studies were included in the review. 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. From the review findings, the authors propose a number of 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) paediatric patients presenting with a sixth cranial nerve palsy require 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. Due to lacking evidence for optimal treatment mechanisms, multi-institutional and international collaborations are suggested as a critical step for future research.
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