Idiopathic intracranial hypertension: a step change in understanding the disease mechanisms
Yiangou, Andreas Mollan, Susan P Sinclair, Alexandra J
Yiangou, Andreas
Mollan, Susan P
Sinclair, Alexandra J
Abstract
The understanding of idiopathic intracranial hypertension (IIH) has evolved over the past few years. Previously, IIH was considered a disease exclusively affecting the neuro-ophthalmic axis, characterized by raised intracranial pressure, headache and papilloedema, and resulting in the risk of severe and permanent visual loss and life-changing disabling headaches. Recent advances have begun to redefine IIH as a probable metabolic disease involving a range of systemic manifestations. More than 95% of individuals affected by the disease are women of reproductive age with obesity. The incidence is rapidly rising and parallels the escalating worldwide obesity rates. Contemporary insights identify associations with insulin resistance, type 2 diabetes and a twofold increased risk of cardiovascular disease in excess of that driven by obesity alone. Adipose distribution in people with IIH, like that in other metabolic diseases, is preferentially centripetal and is associated with changes in intracranial pressure. Evidence now demonstrates adipose tissue dysfunction in people with IIH, involving transcriptional and metabolic priming for lipogenesis and weight gain. Hormonal perturbations are also observed, including a unique phenotype of androgen excess that promotes cerebrospinal fluid secretion. Knowledge of these additional disease features is driving research into novel therapeutic targets and altering the approach to multidisciplinary care.
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Date
2023-11-13
Type
Article
Subject
Endocrinology, Oncology. Pathology., Diseases & disorders of systemic, metabolic or environmental origin
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Citation
Yiangou A, Mollan SP, Sinclair AJ. Idiopathic intracranial hypertension: a step change in understanding the disease mechanisms. Nat Rev Neurol. 2023 Dec;19(12):769-785. doi: 10.1038/s41582-023-00893-0. Epub 2023 Nov 13. PMID: 37957260.
Journal / Source Title
Nature Reviews Neurology
DOI
10.1038/s41582-023-00893-0
PMID
37957260
Publisher
Nature Research