Calcitonin gene related peptide monoclonal antibody treats headache in patients with active idiopathic intracranial hypertension

Andreas Yiangou,James L Mitchell,Edward Bilton,Julie Edwards,Alexandra J Sinclair,Susan P Mollan,Olivia Grech,Vivek Vijay,Gareth G Lavery,Claire Fisher

doi:10.1186/s10194-020-01182-7

Abstract

BackgroundHeadache is the dominant factor for quality of life related disability in idiopathic intracranial hypertension (IIH) and typically has migraine-like characteristics. There are currently no evidence-based therapeutics for headache in IIH, and consequently this is an important unmet clinical need.Case seriesWe report a series of seven patients in whom headaches were the presenting feature of IIH and the headaches had migraine-like characteristics, as is typical in many IIH patients. Papilloedema settled (ocular remission) but headaches continued. These headaches responded markedly to erenumab, a monoclonal antibody targeted against the calcitonin gene related peptide (CGRP) receptor. Of note, there was a recurrence of raised ICP, as evidenced by a return of the papilloedema, however the headaches did not recur whilst treated with erenumab.ConclusionsThose with prior IIH who have their headaches successfully treated with CGRP therapy, should remain under close ocular surveillance (particularly when weight gain is evident) as papilloedema can re-occur in the absence of headache. These cases may suggest that CGRP could be a mechanistic driver for headache in patients with active IIH.

Highlights

Idiopathic intracranial hypertension (IIH) is a chronic debilitating disease characterised by raised intracranial pressure (ICP) that typically occurs in young, obese women [1]
We report seven patients who presented with headaches in the setting of raised ICP and who met the diagnostic criteria for IIH [6], whose IIH remitted and headaches persisted
Female with IIH in occular remission and chronic moderate/severe headaches (≥15 per month) that ≥3 prior conventional oral preventative treatments had failed and suffered recurrence of papilloedema whilst on erenumab. In this cohort treatments that failed included tricyclic antidepressants, topiramate, propranolol, pizotifen, duloxetine, dosulepin and candesartan. These chronic migraine-like headaches were elibigle for treatment with the calcitonin gene related peptide (CGRP) monoclonal antibody erenumab (Aimovig®, Novartis) within the National Health Service (NHS) headache service at University Hospital Birmingham NHS Foundation Trust, UK as part of a free of charge scheme (Novartis)

Summary

Introduction

Idiopathic intracranial hypertension (IIH) is a chronic debilitating disease characterised by raised intracranial pressure (ICP) that typically occurs in young, obese women [1]. Case 2 A 34 year old woman initially presented with increased debilitating headaches, blurred vision, visual obscurations, tinnitus and was noted to have papilloedema by the optometrist. Case 3 A 46 year old woman presented with substantially increased headache symptoms, with migraine-like characteristics, and was noted to have papilloedema.

Results

Conclusion