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Optical coherence tomography for the diagnosis and monitoring of idiopathic intracranial hypertension


The objectives of the study were to investigate the value of optical coherence tomography in detecting papilledema in patients with idiopathic intracranial hypertension (IIH), a disease which is difficult to monitor and which can lead to permanent visual deficits; to analyze retinal changes over time. In this non-interventional case–control study, spectral-domain optical coherence tomography (SD-OCT) was used to analyze the retinal and optic nerve head (ONH) morphology of 21 patients with IIH and 27 age- and sex-matched healthy controls over time. We analyzed the ONH volume using a custom-made algorithm and employed semi-automated segmentation of macular volume scans to assess the macular retinal nerve fiber layer (RNFL) and ganglion cell layer and inner plexiform layer complex as well as the total macular volume. In IIH patients, the ONH volume was increased and correlated with cerebrospinal fluid (CSF) pressure. The ONH volume decreased after the initiation of treatment with acetazolamide. The macular RNFL volume decreased by 5% in 3.5 months, and a stepwise multivariate regression analysis identified CSF pressure as the main influence on macular RNFL volume at diagnosis. The only factor predicting macular RNFL volume loss over time was ONH volume. SD-OCT can non-invasively monitor changes in retinal and ONH morphology in patients with IIH. Increased ONH volume leads to retinal atrophy in the form of macular RNFL volume loss, presumably due to mechanic jamming of the optic nerve at the disc and subsequent axonal loss.

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The authors would like to thank Cheryl Ernest for proofreading.

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Authors and Affiliations



PA conceived the study, performed data acquisition and analysis and drafted the manuscript. CB performed data acquisition and analysis, revised the manuscript for intellectual content and created the artwork. MR performed data acquisition and analysis and revised the manuscript for intellectual content. A-KM performed data acquisition and analysis and revised the manuscript. E-MK performed data analysis and revised the manuscript. DF performed data acquisition and revised the manuscript for intellectual content. RG revised the manuscript for intellectual content. OA revised the manuscript for intellectual content and helped with the interpretation of the data. H-PH revised the manuscript for intellectual content and helped with the interpretation of the data. FP revised the manuscript for intellectual content and helped with the interpretation of the data. AB performed data analysis, revised the manuscript for intellectual content and helped with the interpretation of the data. AM drafted and revised the manuscript for intellectual content and interpreted the data.

Corresponding author

Correspondence to Philipp Albrecht.

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The authors received no funding for the study.

Conflicts of interest

Philipp Albrecht received research grants from Novartis, Biogen Idec, Teva, Merz Pharmaceuticals and travel/accommodation/meeting expenses from Novartis, Teva, Biogen Idec, Merz Pharmaceuticals, Ipsen, Esai and GlaxoSmithKline. Christine Blasberg declares no conflicts of interest. Marius Ringelstein received speaker honoraria from Novartis and Bayer Vital GmbH and travel reimbursement from Bayer Schering and Biogen Idec. Ann-Kristin Müller received travel grants from Novartis. Ella-Maria Kadas declares nothing to disclose. David Finis received speaker honoraria and travel grants from Thea-Pharma, TearScience and Oculus. David Finis is now an employee of Bayer Pharma AG, Wuppertal, Germany. Rainer Guthoff received speaker honoraria and travel/accommodation/meeting expenses from Novartis and Bayer Schering. Orhan Aktas received grants from the German Research Foundation (DFG), Eugène Devic European Network (EU-FP7), German Ministry of Education and Research, Schaufler Foundation, honoraria for lectures from Almirall, Novartis, Bayer, Genzyme, Teva, Merck Serono, Biogen, Roche and Medimmune, and received travel/accommodation/meeting expenses from Novartis, Bayer Schering and Merck Serono. Hans-Peter Hartung received grants from the Walter and Ilse Rose Foundation, the Eugène Devic European Network (EU-FP7) and the German Ministry of Education and Research, received honoraria for consultancy from Bayer Health Care, Biogen Idec, Geneuro, Genzyme, Medimmune, Novartis, Opexa, Receptos, Teva, Sanofi Aventis, and Hoffman La Roche and holds patents. Friedemann Paul received research support, travel grants and personal compensation for activities with Bayer, Teva, Sanofi Genzyme, Merck Serono, Biogen and Novartis. He is supported by the German Research Foundation (DFG Exc 257), the Germany Ministry of Education and Research (BMBF Competence Network Multiple Sclerosis), the European Framework Program (EU-FP7, and the Guthy Jackson Charitable Foundation. Alexander U. Brandt has received consulting fees and speaker honoraria from Novartis, Biogen, Bayer, Teva, Motognosis and Heidelberg Engineering. He is named as co-inventor on a patent application encompassing the optic nerve head volume algorithm. Axel Methner received research and travel grants from Biogen Idec, Novartis and TEVA Pharma.

Ethical standards

The local ethics committee of Heinrich Heine University Düsseldorf approved this prospective observational study. Written informed consent was obtained from all participants in accordance with the Declaration of Helsinki.

Electronic supplementary material

Below is the link to the electronic supplementary material.


Supplemental Figure: Peripapillary Ring Scans in IIH patients. (A/B) Box-and-whisker plots of the mean baseline values for the retinal nerve fiber layer thickness (RNFL) and the total retinal thickness (TRT), measured in peripapillary ring scans centered on the optic disc. The analysis revealed a significant reduction of peripapillary RNFL thickness and TRT in IIH patients compared with healthy controls. (C/D) Box-and-whisker plots of the baseline values and the first follow-up visit (visit 1) as well as p-values for their comparison (GEE analysis) are provided for the peripapillary RNFL and TRT. The investigation only includes those IIH patients who performed a follow-up measurement which was of sufficient quality for analysis: Peripapillary RNFL thickness as well as TRT were significantly reduced in IIH patients at follow-up compared to the baseline. (E/F) Box-and-whisker plots of the mean rates of change per month (Δμm/month) for the different retinal intervals and retinal parameters. Bars in the boxes mark the median, boxes the interquartile range and whiskers minimum and maximum. Numbers of eyes are indicated. P-values for differences for comparison between the IIH patients and healthy controls (A-B), between the baseline and visit 1 (C-D) and between the intervals (E–F) are indicated (bar, GEE analysis) as well as the P-values for differences to zero (E–F, bar, GEE analysis; below the boxes, one-sample t test of left eyes) (TIFF 190 kb)

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Albrecht, P., Blasberg, C., Ringelstein, M. et al. Optical coherence tomography for the diagnosis and monitoring of idiopathic intracranial hypertension. J Neurol 264, 1370–1380 (2017).

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  • Idiopathic intracranial hypertension
  • Optical coherence tomography