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. 2023 Feb;270(2):851-863.
doi: 10.1007/s00415-022-11402-6. Epub 2022 Oct 15.

The idiopathic intracranial hypertension prospective cohort study: evaluation of prognostic factors and outcomes

Mark Thaller  1   2   3 , Victoria Homer  4 , Yousef Hyder  1   2   3 , Andreas Yiangou  1   2   3 , Anthony Liczkowski  1   5 , Anthony W Fong  6   7 , Jasvir Virdee  6 , Rachel Piccus  6 , Marianne Roque  6 , Susan P Mollan  1   6 , Alexandra J Sinclair  8   9   10
Affiliations

The idiopathic intracranial hypertension prospective cohort study: evaluation of prognostic factors and outcomes

Mark Thaller et al. J Neurol. 2023 Feb.

Abstract

Background: There are limited longitudinal data evaluating outcomes in idiopathic intracranial hypertension (IIH). We aimed to evaluate the long-term outcomes in a real-world cohort of patients with IIH and sought to establish the prognostic factors.

Methods: A longitudinal prospective cohort study was conducted over 9 years (2012-2021). Data included demographics and disease status. All consenting patients with IIH were recruited. Visual outcomes included visual acuity, Humphrey visual field and optical coherence tomography (OCT) imaging measurements. Headache frequency, severity, and impact were noted. We analysed the key variables impacting visual and headache outcomes.

Results: The cohort contained 490 patients with a confirmed IIH diagnosis. 98% were female with a mean body mass index (BMI) of 38 kg/m2. Those with the highest OCT retinal nerve fibre layer had the worst visual outcomes. We noted a delayed decline, in the visual field and OCT ganglion cell layer after 12 months. In the medically managed cohort (n = 426), we found that disease duration and change in BMI had the greatest influence on visual outcomes. There was a high burden of headache, with a daily headache at presentation and prior migraine history influencing long-term headache prognosis.

Conclusions: There is a delayed decline in visual outcomes in those with the most severe papilloedema. Disease duration and change in BMI were the key visual prognostic factors, therefore those with the more acute disease may require closer monitoring. Improving prognosis in IIH should focus on the potentially modifiable factor of weight management.

Keywords: Headache; Idiopathic Intracranial Hypertension; Optical coherence tomography; Outcome; Prognosis; Pseudotumor cerebri; Vision.

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Conflict of interest statement

MT, VH, YH, AL, AF, JV, RP, MR report no conflicts. AY reports speaker fees for an educational talk from Teva, UK. SPM reports consultancy fees (Invex Therapeutics; Neurodiem); advisory board fees (Invex therapeutics; Janssen) and speaker fees (Heidelberg engineering; Chugai-Roche Ltd; Allergan; Santen; Chiesi; and Santhera). AJS reports personal fees from Invex therapeutics during the conduct of the study as well as share option and shareholdings, speaker fees (Novartis; Allergan; Teva UK) and consulting fees (Allergan; Chiesi; Novartis; Lundbeck).

Figures

Fig. 1
Fig. 1
CONSORT diagram
Fig. 2
Fig. 2
a Longitudinal data of Humphrey visual field perimetric mean deviation (PMD) in IIH categorised by peak global retinal nerve fibre layer thickness, and LOESS smoothers added to show trends across the categories. b Longitudinal data of macular ganglion cell layer (GCL) volume in IIH categorised by peak global retinal nerve fibre layer thickness, and LOESS smoothers added to show trends across the categories. c Longitudinal data of Humphrey visual field perimetric mean deviation (PMD) in IIH categorised by surgical intervention or not, and LOESS smoothers added to show trends across the categories. d Longitudinal data of macular ganglion cell layer (GCL) volume in IIH categorised by surgical intervention or not, and LOESS smoothers added to show trends across the categories
Fig. 3
Fig. 3
Longitudinal visual data from medically managed IIH patients categorised by disease status—active disease (papilloedema present at enrolment) versus ocular remission (no papilloedema at enrolment), and LOESS smoothers added to show trends across the categories. a LogMAR visual acuity (log units). b Perimetric mean deviation measured by Humphrey visual field 24–2 testing (dB). c Retinal nerve fibre layer thickness measured on Optical Coherence Tomography (µm). d Total retinal thickness of optic nerve head measured on Optical Coherence Tomography (µm). e Macular ganglion cell layer volume measured on Optical Coherence Tomography (mm3)
Fig. 4
Fig. 4
Longitudinal headache data from medically managed IIH patients with active disease (papilloedema present at enrolment), and LOESS smoothers added to show trends across the categories. a Headache frequency (days per month). b Migraine-like headache frequency (days per month). c Headache mean severity of predominant headache (0–10 numerical rating scale). d Headache Impact Test 6 (HIT6) (quality of life measure score 36–78)
See this image and copyright information in PMC

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