Idiopathic Intracranial Hypertension

Matthew J. Thurtell, MBBS, MSc, FRACP Neuro-ophthalmology p. 1289-1309 October 2019, Vol.25, No.5 doi: 10.1212/CON.0000000000000770
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PURPOSE OF REVIEW Idiopathic intracranial hypertension is a syndrome of increased intracranial pressure of unclear etiology that most often occurs in obese women of childbearing age but can also occur in men, children, and older adults. This article reviews the diagnostic criteria, clinical features, neuroimaging findings, differential diagnosis, and management options for this condition.

RECENT FINDINGS Recent population studies have found that the annual incidence of idiopathic intracranial hypertension is increasing in association with obesity rates, whereas recent scientific studies indicate a possible role for androgen sex hormones and adipose tissue in the pathogenesis of the disease. Prospective clinical trials have demonstrated a role for weight loss, acetazolamide, and topiramate in the management of mild disease. A recently begun randomized multicenter trial of surgical interventions will provide insight into the indications for surgical intervention, optimal timing and choice of intervention, and long-term outcomes.

SUMMARY Idiopathic intracranial hypertension is a disorder producing symptoms and signs of increased intracranial pressure in the absence of an alternative cause. The main goals of treatment are to preserve visual function and alleviate symptoms, which can usually be achieved with a combination of weight loss, medical therapies, and surgical interventions depending on the severity of symptoms and vision loss, response to treatment, and subsequent clinical course.

Address correspondence to Dr Matthew J. Thurtell, University of Iowa Hospitals & Clinics, 200 Hawkins Dr, Pomerantz Family Pavilion, Iowa City, IA 52242, [email protected].

RELATIONSHIP DISCLOSURE: Dr Thurtell serves on the editorial board of the Journal of Neuro-Ophthalmology, receives research/grant support from the National Eye Institute (U10-EY025990), and receives book royalties from Oxford University Press.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Thurtell discusses the unlabeled/investigational use of acetazolamide, furosemide, methazolamide, and topiramate for the treatment of idiopathic intracranial hypertension.

© 2019 American Academy of Neurology.