Headache Attributed to Idiopathic Intracranial Hypertension and Persistent Post-idiopathic Intracranial Hypertension Headache

A Narrative Review

Susan P. Mollan FRCOphth; Olivia Grech MRes; Alexandra J. Sinclair PhD

Disclosures

Headache. 2021;61(6):808-816. 

In This Article

Abstract and Introduction

Abstract

Objective: Headache is a near-universal sequela of idiopathic intracranial hypertension (IIH). The aim of this paper is to report current knowledge of headache in IIH and to identify therapeutic options.

Background: Disability in IIH is predominantly driven by headache; thus, headache management is an urgent and unmet clinical need. At present, there is currently no scientific evidence for the directed use of abortive or preventative headache therapy.

Methods: A detailed search of the scientific literature and narrative review was performed.

Results: Headache in IIH is driven by raised intracranial pressure (ICP) and reduction of ICP has been reported in some studies to reduce headache. Despite resolution of papilledema and normalization of raised ICP, a majority suffer persistent post-IIH headache. The lack of evidence-based management approaches leaves many untreated. Where clinicians attempt to manage IIH headache, they use off-label therapies to target the prevailing headache phenotype. A recent prospective open-label study demonstrated the effective use of a calcitonin gene-related peptide monoclonal antibody therapy in IIH for persistent post-IIH headache.

Conclusions: There is overwhelming evidence of the headache burden in IIH. Studies are required to investigate the biological foundations of headache related to ICP and to develop treatments specifically directed to manage headache in IIH.

Introduction

Idiopathic intracranial hypertension (IIH) is a disabling secondary headache disorder characterized by raised intracranial pressure (ICP) and papilledema with the potential risk of permanent visual loss.[1,2] There is an established association with obesity[3,4] and increasing prevalence[5–7] currently estimated at 68 per 100,000 females.[6] Although there has been progress in diagnostic algorithims,[8–11] there are little data to help guide treatment decisions for headache.[9,12]

IIH has a detrimental effect on all aspects of the patient's quality of life,[13,14] predominantly driven by headache.[14] Clinical trials report a substantial headache burden,[15–17] and there are no directed trials investigating headache therapies. The onset of headaches attributable to IIH is temporally related to increased ICP, which improve in response to reduction of ICP.[18] Following resolution of papilledema, those with persistent post-IIH headache frequently have migraine-like characteristics[15–17] contributing to long-term morbidity.[19] Patients and physicians have ranked headache as a high research priority.[20] The aim of this narrative review is to describe the progress in understanding of headache attributed to IIH and to generate momentum to identify effective therapies.

Comments

3090D553-9492-4563-8681-AD288FA52ACE

processing....