Combined Idiopathic Intracranial Hypertension score, an integrated severity and treatment-oriented tool for idiopathic intracranial hypertension: a retrospective cohort study
Abstract
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.
Related
- Regional Perivascular Space Burden in Pediatric Idiopathic Intracranial Hypertension: A Case–Control MRI Study
- Factors Associated with Subsequent Lumboperitoneal Shunt Requirement in Adult Women with Idiopathic Intracranial Hypertension: A Prospective Cohort Study
- Longitudinal Optical Coherence Tomography Indices in Pediatric Idiopathic Intracranial Hypertension
- Bedside Transcranial Doppler as a Dynamic Warning Tool for Rapidly Evolving Intracranial Hypertension in Pneumococcal Meningitis
- External Lumbar Drainage for Refractory Intracranial Hypertension in Acute Brain Injury: A Narrative Review and Case Series
BibTeX
@article{villain2026combined,
title = {{Combined Idiopathic Intracranial Hypertension score, an integrated severity and treatment-oriented tool for idiopathic intracranial hypertension: a retrospective cohort study}},
author = {Max Villain and Marie Latypov and Eloi Debourdeau and Pierre Meyer and Audrey de Jong and Valentin Favier and Florent Fuchs and Luc Jeanjean and Cyril Dargazanli and Chloé Chamard and Anne Ducros and Frédéric Michon and Vincent Costalat and Vincent Daïen and Federico Cagnazzo},
journal = {Journal of Headache and Pain},
year = {2026},
month = sep,
doi = {10.1186/s10194-026-02501-0},
url = {https://hal.science/hal-05755540v1},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Combined Idiopathic Intracranial Hypertension score, an integrated severity and treatment-oriented tool for idiopathic intracranial hypertension: a retrospective cohort study
AU - Max Villain
AU - Marie Latypov
AU - Eloi Debourdeau
AU - Pierre Meyer
AU - Audrey de Jong
AU - Valentin Favier
AU - Florent Fuchs
AU - Luc Jeanjean
AU - Cyril Dargazanli
AU - Chloé Chamard
AU - Anne Ducros
AU - Frédéric Michon
AU - Vincent Costalat
AU - Vincent Daïen
AU - Federico Cagnazzo
PY - 2026
DA - 2026/09/09
JO - Journal of Headache and Pain
DO - 10.1186/s10194-026-02501-0
UR - https://hal.science/hal-05755540v1
AB - 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.
SN - 1129-2369
SN - 1129-2377
ER -