Are the ICHD-3 criteria for headache attributed to idiopathic intracranial hypertension valid? Headache phenotyping and field-testing in newly diagnosed idiopathic intracranial hypertension.

Background: Headache burden is substantial in idiopathic intracranial hypertension. The classification of idiopathic intracranial hypertension headache by the International Classification of Headache Disorders (ICHD) is an important tool for research and clinical purposes.
Methods: We phenotyped headaches and tested sensitivity and specificity of the ICHD-3 criteria for idiopathic intracranial hypertension headache in a prospective cohort of patients suspected of idiopathic intracranial hypertension at two tertiary headache centers.
Results: Sensitivity was 93% and specificity was 100% of ICHD-3 criteria for idiopathic intracranial hypertension-related headache validated in idiopathic intracranial hypertension ( n  = 140) and patients in whom idiopathic intracranial hypertension was suspected but disproven ( n =  103). The phenotype of new/worsened headaches related to idiopathic intracranial hypertension suspicion was equally migraine-like (p = 0.76) and tension-type-like (p = 0.08). Lumbar puncture opening pressure was higher ( p  < 0.0001) and pulsatile tinnitus more frequent ( p  < 0.0001) in idiopathic intracranial hypertension patients, but neither improved the applicability of the headache criteria, nor did papilledema.
Conclusion: Headache phenotype is not distinct in idiopathic intracranial hypertension. ICHD-3 criteria for idiopathic intracranial hypertension headache are sensitive and specific, but simplicity can be improved without compromising accuracy. We propose that a new or worsened headache temporally related to active idiopathic intracranial hypertension is a sufficient criterion for idiopathic intracranial hypertension headache regardless of headache phenotype or accompanying symptoms, and that elements of idiopathic intracranial hypertension diagnostics (papilledema and opening pressure) be segregated from headache criteria. Trial Registration: ClinicalTrials.gov Identifier: NCT04032379.
Competing Interests: Declaration of conflicting interestsThe authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: NSH received funding from the Novo Nordic Foundation during the conduction of the work and gave lectures for Pfizer, University of Southern Denmark, and University of Copenhagen. JJK received funding from the Lundbeck Foundation, Rigshospitalet-Glostrup, and Odense University hospital for the duration of the study. HMY received support from Abbvie for participation in non-related courses. RHJ gave lectures for Pfizer, Eli-Lilly, Merck, TEVA, Novartis, Lundbeck and Allergan; was investigator in clinical trials with Eli-Lilly, Novartis and Lundbeck. She is the Director of Danish Headache Center, Lifting The Global Burden of Headache and Founder of Master of Headache Disorders at University of Copenhagen and received research funding from University of Copenhagen, Rigshospitalet, Lundbeck Foundation, The Medical Society in Copenhagen, NovoNordisk Foundation and Tryg Foundation. DB gave lectures for TEVA, Novartis, Pfizer. Received travel support from Allergan, TEVA, Pfizer, Abbvie; was in advisory boards for Novartis, Lilly, Teva, Lundbeck, Pfizer, Abbvie and participated in clinical trials for TEVA, Lundbeck, Novartis, Lilly, Novo Nordic Foundation outside the submitted work. Received grants from Odense University Hospital and Rigshospitalet, University of Copenhagen during the conduction of the work.