Reviewing the Recent Developments in Idiopathic Intracranial Hypertension

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Reviewing the Recent Developments in Idiopathic Intracranial Hypertension Jasvir Virdee Alex J. Sinclair

. Stephanie Larcombe

. Vivek Vijay .

. Margaret Dayan . Susan P. Mollan

Received: February 19, 2019 Ó The Author(s) 2020

ABSTRACT There is increasing evidence and appreciation of idiopathic intracranial hypertension (IIH) in medicine. The pathological processes underlying raised intracranial pressure are being studied, with new insights found in both hormonal dysregulation and the metabolic neuroendocrine axis. These will potentially lead to novel therapeutic targets for IIH. The first consensus

guidelines have been published on the investigation and management of adult IIH, and the International Headache Society criteria for headache attributable to IIH have been modified to reflect our evolving understanding of IIH. Randomized clinical trials have been published, and a number of studies in this disease area are ongoing.

Keywords: Headache; Idiopathic intracranial hypertension; Lumbar puncture; Obesity; Papilloedema; Pseudotumour cerebri; Raised ICP; Weight management Digital Features To view digital features for this article go to https://doi.org/10.6084/m9.figshare.12547667 Jasvir Virdee and Stephanie Larcombe are joint first authors. J. Virdee Ophthalmology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK S. Larcombe Medical School, University of Birmingham, Birmingham, UK V. Vijay  A. J. Sinclair Metabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK

V. Vijay  A. J. Sinclair Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK A. J. Sinclair  S. P. Mollan (&) Birmingham Neuro-Ophthalmology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK e-mail: [email protected] M. Dayan Newcastle Eye Centre, The Newcastle Upon Tyne Hospitals NHS Foundation Trust, Royal Victoria Infirmary, Newcastle upon Tyne, UK

Ophthalmol Ther

Key Summary Points Why carry out this study? Investigation and management strategies have been recommended by the first consensus guidance for IIH. Understanding the underlying pathophysiology of IIH is identifying novel avenues for targeted therapies. What was learned from the study? This review summarizes the IIH pathophysiology known to date. It presents an evaluation of the randomized controlled trials in IIH. It details the potential targets for future disease management.

DIGITAL FEATURES This article is published with digital features to facilitate understanding of the article. To view digital features for this article go to https://doi. org/10.6084/m9.figshare.12547667.

INTRODUCTION Idiopathic intracranial hypertension (IIH), also known as primary pseudotumour cerebri syndrome, is a neurological disorder characterised by raised intracranial pressure (ICP) that can cause papilloedema with no identifiable cause found [1, 2]. There is a rising incidence of this disease, which is m