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

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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.

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Notes

  1. At later stages optic nerve atrophy may become more prominent and is structurally related to RNFL and GCL loss on retinal OCT.

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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Acknowledgments

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

None.

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Authors and Affiliations

  1. Moorfields Eye Hospital, The National Hospital for Neurology and Neurosurgery and St. Thomas’ Hospital, London, UK

    Gordon T. Plant

  2. Department of Neurology, VU University Medical Center, De Boelelaan 1117, 1081 HV, Amsterdam, The Netherlands

    Axel Petzold

  3. UCL Institute of Neurology, Neuroimmunology and CSF Laboratory, Queen Square, London, UK

    Axel Petzold

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  1. Axel Petzold
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  2. Gordon T. Plant
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Correspondence to Axel Petzold.

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Petzold, A., Plant, G.T. Chronic relapsing inflammatory optic neuropathy: a systematic review of 122 cases reported. J Neurol 261, 17–26 (2014). https://doi.org/10.1007/s00415-013-6957-4

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