Combined Idiopathic Intracranial Hypertension score, an integrated severity and treatment-oriented tool for idiopathic intracranial hypertension: a retrospective cohort study

IIH has evolved into a multidisciplinary condition managed across neurology, ophthalmology, interventional neuroradiology, and surgery. Existing tools address only partial aspects of disease severity; no unified, treatment-oriented classification exists to support multidisciplinary decision-making. Cross-specialty communication is inconsistent leading to heterogenous therapeutic decisions. We aimed to develop and evaluate a standardized multidisciplinary score for idiopathic intracranial hypertension (IIH) and assess its association with therapeutic escalation. We enrolled adults fulfilling the revised Friedman criteria at a tertiary interventional neuroradiology referral center from 2020 to 2025. Clinical data were organized into four domains: ophthalmologic severity with a combination of visual function and tomographic data (E grades 1–4), neuroradiology findings (N grade A to C), intracranial pressure (ICP), and treatment modality. We included 250 patients (93.0% female, median age at diagnosis 29.0 years, median ICP 34.0 cmH 2 O). E grade was significantly associated with invasive intervention ( p < 0.001 for endovascular stenting, Cochran-Armitage test for trend). Among E1-grade patients, 64% underwent endovascular stenting and 64% received high-dose methylprednisolone. Firth’s penalized logistic regression confirmed E grade as a significant independent predictor of invasive interventions (adjusted odds ratio = 0.47 per grade [toward milder disease]; 95% CI 0.32–0.68, p < 0.001). Inter-rater agreement for E grading was substantial (weighted Cohen’s κ 0.82, 95% CI 0.68–0.96). The Combined Idiopathic Intracranial Hypertension score provides a shared, notation for multidisciplinary IIH documentation. In this cohort, the ophthalmologic grade and intracranial pressure were associated with the need for invasive intervention (adjusted OR 0.47 per grade toward milder disease; AUC 0.68), and E grading showed substantial inter-rater agreement (weighted κ 0.82). Prospective external validation is required before the complete four-domain score can be recommended for clinical decision-making. Not applicable.

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Journal
The Journal of Headache and Pain
Published
2026-09-09
DOI
https://doi.org/10.1186/s10194-026-02501-0
Primary Topic
Cerebral Venous Sinus Thrombosis
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article
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article

Combined Idiopathic Intracranial Hypertension score, an integrated severity and treatment-oriented tool for idiopathic intracranial hypertension: a retrospective cohort study

Vincent Daïen, Vincent Costalat, Cyril Dargazanli, Frédéric Michon et al.
The Journal of Headache and Pain
Cerebral Venous Sinus Thrombosis
article

Combined Idiopathic Intracranial Hypertension score, an integrated severity and treatment-oriented tool for idiopathic intracranial hypertension: a retrospective cohort study

Vincent Daïen, Vincent Costalat, Cyril Dargazanli, Frédéric Michon, Chloé Chamard, Valentin Favier, Pierre Meyer, Eloi Debourdeau, Marie Latypov, Anne Ducros, L. Jeanjean, Max Villain, Federico Cagnazzo, Florent Fuchs, Audrey De Jong
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The Journal of Headache and Pain
The University of Sydney (AU), Inserm (FR), Université de Montpellier (FR), Centre Hospitalier Universitaire de Montpellier (FR), Hôpital Gui de Chauliac (FR), Institute for Neurosciences of Montpellier (FR), Université de Nîmes (FR)
Peace, Justice and strong institutions
Openalex Percentile: Top 11%
Cerebral Venous Sinus Thrombosis
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