Risk factors and disease associations in people living with idiopathic intracranial hypertension

ABSTRACT

Introduction

Idiopathic intracranial hypertension is a neurological condition characterized by a raised intracranial pressure and papilledema, leading to chronic headaches and visual disturbances. By recognizing modifiable risk factors and deploying evidence-based interventions, healthcare providers have the potential to mitigate the burden of the disease and improve patient outcomes.

Areas covered

It is well known that the condition occurs in predominantly pre-menopausal females who live with obesity particularly in the context of recent weight gain. This review discusses what risk factors may contribute to the metabolic underpinnings of cerebrospinal fluid dysregulation. There are a number of disease associations that are important to screen for as they can alter management.

Expert opinion

There is emerging evidence to suggest that idiopathic intracranial hypertension is a systemic metabolic disease and it is unknown what are all the risk factors are that precipitate the condition. Targeting certain hardwired risk factors is unachievable. However, as recent weight gain has been identified as a predominant risk factor for the development of the disease and relapse, modification of body weight should be the primary aim of management. Insightful research into the involvement of the neuroendocrine axis driving cerebrospinal fluid dysregulation now has the potential for the development of therapeutic targets.

KEYWORDS:

• Cerebrospinal fluid
• female sex
• Glucocorticoid dysregulation intracranial pressure
• obesity
• papilledema
• risk factor
• weight loss

Article highlights

• Idiopathic intracranial hypertension is a systemic metabolic disease where living with obesity and recent weight gain appear to be risk factors.

• Hardwired risk factors that are associated with a diagnosis of IIH and include female gender and those of reproductive age, and these may provide insights to disease pathogenesis.

• Understanding how to modulate cerebrospinal fluid secretion, a key driver for signs and symptoms of idiopathic intracranial hypertension, has led to the repurposing of two targeted therapies namely an 11β-Hydroxysteroid dehydrogenase type 1 inhibitor, AZD4017, and a glucagon-like peptide-1 receptor agonist, Exentide.

• The impact of idiopathic intracranial hypertension as a systemic metabolic disease confers a heightened risk of future cardiac disease, may lower fertility and induce pregnancy complications of pre-eclampsia, gestational diabetes and delivery complications. All of these findings have been found to be unique to idiopathic intracranial hypertension as the studies have meticulous used control populations that are living with obesity without idiopathic intracranial hypertension.

• Future work needs to continue to unpick the underlying pathogenesis to address the unknown factors that may be relevant to this condition.

Declaration of interest

S Mollan has received payment for consultancy work from Invex Therapeutics. She has received payment for advisory boards from Genentech and Ocular Therapeutix. Grant funding has been paid to her institution from the National Institute of Health Research (NIHR131211), the UK Space Agency and IIH UK. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.