Journal of Neurology

, Volume 261, Issue 1, pp 17–26 | Cite as

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

  • Axel PetzoldEmail author
  • Gordon T. Plant


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.


Optic neuritis Steroid-dependent optic neuritis Relapse ON CRION 

List of abbreviations


Anterior ischemic optic neuropathy




Count fingers


Chronic relapsing inflammatory optic neuropathy


Cerebrospinal fluid


Computed tomography


Giant cell arteritis


Ganglion cell layer


Glial fibrillary acidic protein


Hand movement


Inner nuclear layer


Isolated optic neuritis


Intravenous immunoglobulin


Intravenous methylprednisolone


Leber’s hereditary optic neuropathy


Lumbar puncture


Microcystic macular edema


Magnetic resonance imaging


Multiple sclerosis


Multiple sclerosis optic neuritis


Neuromyelitis optica


No light perception


Optical coherence tomography


Optic neuritis


Optic neuritis treatment trial


Dominant optical atrophy


Plasma exchange


Posterior ischemic optic neuropathy


Relapsing isolated optic neuritis


Retinal nerve fiber layer


Relapsing remitting multiple sclerosis


Systemic lupus erythematosus


Unclassified optic neuritis


Visual acuity



The MS Center VUMC is partially funded by a program grant of the Dutch MS Research Foundation. We are grateful to the following authors for providing additional information on their patients: Desmond Kidd, Aureline Benoilid and Alessandra Falcao. Crion is also the name of a small village between Lunéville and the Parroy Forrest in France, which was miraculously saved from bombing in the First World War.

Conflicts of interest



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Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  1. 1.Moorfields Eye Hospital, The National Hospital for Neurology and Neurosurgery and St. Thomas’ HospitalLondonUK
  2. 2.Department of NeurologyVU University Medical CenterAmsterdamThe Netherlands
  3. 3.UCL Institute of Neurology, Neuroimmunology and CSF LaboratoryLondonUK

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