Journal of Neurology

, Volume 261, Issue 1, pp 17–26

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

Review

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.

Keywords

Optic neuritis Steroid-dependent optic neuritis Relapse ON CRION 

List of abbreviations

AION

Anterior ischemic optic neuropathy

AQP4

Aquaporin-4

CF

Count fingers

CRION

Chronic relapsing inflammatory optic neuropathy

CSF

Cerebrospinal fluid

CT

Computed tomography

GCA

Giant cell arteritis

GCL

Ganglion cell layer

GFAP

Glial fibrillary acidic protein

HM

Hand movement

INL

Inner nuclear layer

ION

Isolated optic neuritis

IVIG

Intravenous immunoglobulin

IVMP

Intravenous methylprednisolone

LHON

Leber’s hereditary optic neuropathy

LP

Lumbar puncture

MMO

Microcystic macular edema

MRI

Magnetic resonance imaging

MS

Multiple sclerosis

MSON

Multiple sclerosis optic neuritis

NMO

Neuromyelitis optica

NLP

No light perception

OCT

Optical coherence tomography

ON

Optic neuritis

ONTT

Optic neuritis treatment trial

OPA

Dominant optical atrophy

PE

Plasma exchange

PION

Posterior ischemic optic neuropathy

RION

Relapsing isolated optic neuritis

RNFL

Retinal nerve fiber layer

RRMS

Relapsing remitting multiple sclerosis

SLE

Systemic lupus erythematosus

UCON

Unclassified optic neuritis

VA

Visual acuity

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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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