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.

Cognitive function in idiopathic intracranial hypertension: a prospective case-control study.

OBJECTIVE: To explore the extent and nature of cognitive deficits in patients with idiopathic intracranial hypertension (IIH) at the time of diagnosis and after 3 months of treatment. DESIGN: Prospective case-control study. SETTING: Neurological department, ophthalmological department and a tertiary headache referral clinic at a Danish university hospital. PARTICIPANTS: 31 patients with definite IIH referred from June 2011 to February 2013 and included within 1 week of diagnostic intracranial pressure (ICP) measurement. 29 patients completed re-examination at the 3-month follow-up. At the time of testing, none of the patients took medication potentially affecting cognitive function. Controls were 31 healthy age-matched and sex-matched volunteers from the local community. OUTCOME MEASURES: Executive function, working memory, visuospatial memory, processing speed, attention and reaction time assessed by a comprehensive neuropsychological test battery consisting of validated computerised (Cambridge neuropsychological test automated battery) and paper-and-pencil tests. RESULTS: Patients with IIH performed significantly worse than controls in four of six cognitive domains (p≤0.02). Deficits were most pronounced in reaction time (1.45 SD below controls 95% CI 2.10 to 0.85) and processing speed (1.48 SD below controls 95% CI 2.08 to 0.81). Despite marked improvement in ICP and headache, re-examination showed persistent cognitive dysfunction 3 months after diagnosis and start of treatment. CONCLUSIONS: We demonstrate for the first time in a well-defined cohort of patients that IIH may be associated with cognitive dysfunction. This could explain the functional disability of patients with IIH. A focused multidisciplinary approach including neuropsychological rehabilitation, therefore, might be relevant in the treatment of patients with IIH.