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

J Neurol. 2014 Jan;261(1):17-26. doi: 10.1007/s00415-013-6957-4. Epub 2013 May 23.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review
  • Systematic Review

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Chronic Disease
  • Databases, Bibliographic / statistics & numerical data
  • Diagnosis, Differential
  • Female
  • Global Health
  • Humans
  • Male
  • Middle Aged
  • Optic Neuritis / complications
  • Optic Neuritis / diagnosis*
  • Optic Neuritis / epidemiology*
  • Optic Neuritis / therapy*
  • Pain / etiology
  • Retrospective Studies
  • Vision Disorders / etiology
  • Visual Acuity
  • Young Adult