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Published in: Journal of Neurology 1/2014

01-01-2014 | Review

Chronic relapsing inflammatory optic neuropathy: a systematic review of 122 cases reported

Authors: Axel Petzold, Gordon T. Plant

Published in: Journal of Neurology | Issue 1/2014

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Abstract

Chronic relapsing inflammatory optic neuropathy (CRION) is an entity that was described in 2003. Early recognition of patients suffering from CRION is relevant because of the associated risk for blindness if treated inappropriately. It seems timely to have a clinical review on this recently defined entity. A systematic literature review, irrespective of language, retrieved 22 case series and single reports describing 122 patients with CRION between 2003 and 2013. We review the epidemiology, diagnostic workup, differential diagnosis, and treatment (acute, intermediate, and long term) in view of the collective data. These data suggest that CRION is a distinct nosological entity, which is seronegative for anti-aquaporin four auto-antibodies and recognized by and managed through its dependency on immuno-suppression. Revised diagnostic criteria are proposed in light of the data compromising a critical discussion of relevant limitations.
Footnotes
1
At later stages optic nerve atrophy may become more prominent and is structurally related to RNFL and GCL loss on retinal OCT.
 
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Metadata
Title
Chronic relapsing inflammatory optic neuropathy: a systematic review of 122 cases reported
Authors
Axel Petzold
Gordon T. Plant
Publication date
01-01-2014
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 1/2014
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-013-6957-4

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