Idiopathic intracranial hypertension (IIH) is a condition of significant morbidity and rising prevalence. It typically affects young people living with obesity, mostly women of reproductive age, and can present with headaches, visual abnormalities, tinnitus and cognitive dysfunction. Raised intracranial pressure without a secondary identified cause remains a key diagnostic feature of this condition, however, the underlying pathophysiological mechanisms that drive this increase are poorly understood. Previous theories have focused on cerebrospinal fluid (CSF) hypersecretion or impaired reabsorption, however, the recent characterisation of the glymphatic system in many other neurological conditions necessitates a re-evaluation of these hypotheses. Further, the impact of metabolic dysfunction and hormonal dysregulation in this population group must also be considered. Given the emerging evidence, it is likely that IIH is triggered by the interaction of multiple aetiological factors that ultimately results in the disruption of CSF dynamics. This review aims to provide a comprehensive update on the current theories regarding the pathogenesis of IIH.
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Contributors BDC: conceptualisation, methodology, writing original draft, reviewing and editing, figure conceptualisation and design. MNLN, SR, PS and OW: reviewing and editing. FB, EJH and JF: reviewing, editing and supervision. AvdW: conceptualisation, writing, reviewing, editing and supervision. All authors contributed to the article and approved the submitted version.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests PS has co-authored manuscripts with medical writing provided by Novo Nordisk and Eli Lilly. She is supported by an Investigator Grant from the National Health and Medical Research Council (1178482). JF receives funding from Genzyme and Biogen and has received honorarium from Novartis. AvdW has received travel support and served on advisory boards for Novartis, Biogen, Merck Serono, Roche and Teva. She receives grant support from the National Health and Medical Research Council of Australia and MS Research Australia.
Provenance and peer review Not commissioned; externally peer reviewed.