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Applying the 2022 optic neuritis criteria to noninflammatory optic neuropathies with optic nerve T2-hyperintensity: an observational study

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Abstract

Introduction

Recent diagnostic criteria for optic neuritis include T2-hyperintensity of the optic nerve (ON), even without associated contrast enhancement. However, isolated ON-T2-hyperintensity is a nonspecific finding found in any optic neuropathy or severe retinopathy. We applied the 2022 optic neuritis diagnostic criteria to a cohort of patients with noninflammatory optic neuropathy and ON-T2-hyperintensity in at least one eye, to assess the rate of optic neuritis misdiagnosis using these criteria.

Methods

Retrospective study of consecutive patients who underwent brain/orbit MRI with/without contrast between 07/01/2019 and 06/30/2022. Patients with ON-T2-hyperintensity in at least one eye were included. The 2022 optic neuritis diagnostic criteria were applied to patients with noninflammatory optic neuropathies who had an ophthalmologic examination available for review.

Results

Of 150 patients included, 85/150 had compressive optic neuropathy; 32/150 had glaucoma; 12/150 had papilledema; 8/150 had hereditary (3), radiation-induced (3), nutritional (1), traumatic (1) optic neuropathies (none fulfilled the criteria); 13/150 had ischemic optic neuropathy and 4 fulfilled the criteria as definite optic neuritis due to contrast enhancement of the ON head. Seven additional patients would have satisfied the diagnostic criteria if red flags for alternative diagnoses had been overlooked.

Discussion

The application of the 2022 optic neuritis diagnostic criteria in patients with noninflammatory optic neuropathy and ON-T2-hyperintensity in at least one ON resulted in misdiagnosis of optic neuritis in only 4 patients because of ON head enhancement, all with nonarteritic anterior ischemic optic neuropathy. Neuro-ophthalmologic evaluation and exclusion of the ON head as a location in the MRI criteria would have prevented optic neuritis misdiagnosis in our study.

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Data availability

Anonymized data not published within this article will be made available by request from any qualified investigator.

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Funding

V. Biousse and N. J. Newman are supported by the National Institutes of Health’s National Eye Institute core Grant P30-EY06360 (Department of Ophthalmology, Emory University School of Medicine) and by a departmental grant from Research to Prevent Blindness (New York, NY). F. Labella received a research grant from Fundación Alfonso Martín Escudero, Madrid, Spain, for clinical research in neuro-ophthalmology. V. Biousse is a consultant for GenSight Biologics and Neurophoenix and receives research support from GenSight Biologics. N. J. Newman is a consultant for GenSight Biologics, Santhera/Chiesi, Stoke, Avidity, and Neurophoenix; receives research support from GenSight Biologics and Santhera/Chiesi; is a participant in educational webinars sponsored by WebMD-Global Medscape.

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All authors contributed to the study conception and design, material preparation, data collection and analysis. The first draft of the manuscript was written by Fernando Labella Álvarez, Valérie Biousse and Nancy J. Newman; all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Nancy J. Newman.

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The authors declare that they have no conflict of interest.

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This study was performed in accordance with the ethical standards of the Declaration of Helsinki. This study was approved by our institutional review board (Emory University Institutional Review Board). The need for informed consent was waived.

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Labella Álvarez, F., Biousse, V., Mosleh, R. et al. Applying the 2022 optic neuritis criteria to noninflammatory optic neuropathies with optic nerve T2-hyperintensity: an observational study. J Neurol (2024). https://doi.org/10.1007/s00415-024-12335-y

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