Optic neuritis

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Last reviewed: 21 Nov 2024

Last updated: 06 Jun 2024

Optic neuritis (ON) may involve the retrobulbar (retrobulbar neuritis) or the intrabulbar (papillitis) portion of the optic nerve, or both.

Idiopathic ON, the most common form of ON, is an inflammatory optic neuropathy in the absence of evidence of a systemic inflammatory disease.

May occur in isolation or be a manifestation, often the first, of multiple sclerosis (MS).

Investigations including MRI of the brain can help predict the risk of conversion to MS and can dictate early intervention with disease-modifying treatments (interferon-beta, glatiramer acetate) that reduces the risk of conversion to MS.

Acute treatment for idiopathic ON includes high-dose oral or intravenous corticosteroid treatment.

Low-to-medium dose oral corticosteroids alone should not be used in the treatment of idiopathic acute ON.

Definition

ON represents inflammation of the optic nerve. By far the most common form is idiopathic ON, which is a primary demyelinating disease occurring in isolation or as part of MS.[1][2] When in isolation, primary demyelinating ON is considered a forme fruste of MS. ON manifests as the subacute or acute onset of pain in the eye and pain with eye movements and loss of vision, typically with central or centrocecal scotoma that usually reaches a nadir at approximately 1-2 weeks. ON recovers over several months, with ultimate visual acuity that is possibly reduced but seldom severely so.

History and exam

Key diagnostic factors

periorbital/retro-ocular pain
loss of visual acuity with scotoma
color desaturation/loss of color vision
relative afferent papillary defect (RAPD)

Full details

Other diagnostic factors

optic disk swelling
neurologic abnormalities of multiple sclerosis
Uhthoff phenomenon
phosphenes
Pulfrich phenomenon
perivenous sheathing

Full details

Risk factors

age 30 to 50 years
female sex
white ethnicity
HLA-DRB1*1501 genotype
risk factors for multiple sclerosis (MS; Epstein-Barr virus [EBV] infection, vitamin D deficiency, and smoking)
childhood in higher latitudes
presence of autoimmune disease
exposure to infectious diseases, such as Lyme disease and syphilis

Full details

Diagnostic tests

1st tests to order

MRI - brain
CBC
ESR
C-reactive protein
venereal disease research laboratory (VDRL)
aquaporin-4 (AQP4) antibody, myelin oligodendrocyte glycoprotein (MOG) antibody, and collapsin response mediator protein 5 (CRMP5) antibody
uric acid
serum ACE
ANA
B12 and folate

Full details

Tests to consider

Lyme titer
cerebrospinal fluid analysis

Full details

Treatment algorithm

ACUTE

acute episode

ONGOING

coexisting inflammatory diseases (e.g., NMOSD, MOGAD, SLE or sarcoidosis)

Contributors

Authors

Cris S. Constantinescu, MD, PhD

Chair of Neurology Emeritus

Academic Unit of Mental Health and Clinical Neuroscience

University of Nottingham

Nottingham

Attending Neurologist

Professor of Neurology

Cooper Neuroscience Institute

Camden

New Jersey

Disclosures

CSC has received research support or consultancy fees from Biogen, GW, Merck, Morphosys, Novartis, Roche, Sanofi-Pasteur, MSD, and Teva. CSC is an author of a number of references cited in this topic.

Thomas M. Bosley, MD

Knights Templar Eye Foundation Professor of Ophthalmology

Wilmer Eye Institute

Johns Hopkins University

Baltimore

Disclosures

TMB declares that he has no competing interests.

Peer reviewers

Gus Gazzard, MA, MBBChir, MD, FRCOphth

Consultant Ophthalmic Surgeon

King's College Hospital

Honorary Research Fellow

Institute of Ophthalmology

London

Disclosures

GG declares that he has no competing interests.

John Selhorst, MD

Professor

Department of Neurology and Psychiatry

Saint Louis University

St. Louis

Disclosures

JS declares that he has no competing interests.

Efstratios Mendrinos, MD

Department of Ophthalmology

Geneva University Hospitals

Geneva

Switzerland

Disclosures

EM declares that he has no competing interests.

Jonathan Smith, MD

Specialist Registrar in Ophthalmology

Royal Victoria Infirmary

Newcastle

Disclosures

JS declares that he has no competing interests.

Peter Sguigna, MD

Assistant Professor

Department of Neurology

The University of Texas

Southwestern Medical Center

Dallas

Disclosures

PS has received grant support from National Multiple Sclerosis Society, International Progressive Multiple Sclerosis Alliance, National Institute of Health (NIH), Center for Translational Medicine, Physician Scientist Training Program, and Department of Defense (DOD). He has received research support from Genentech, Clene Nanomedicine, and Patient Centered Outcomes Research Institute. He has received consulting fees from Medical Logix LLC, Genentech, EMD Serono, Horizon Therapeutics, and Bristol Myers Squibb. He is the inventor on a patent for OCT technology that is held by UT Southwestern Medical Center.

Differentials
- Lyme disease
- Syphilis
- HIV infection
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Guidelines
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Patient information
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Optic neuritis

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic tests

1st tests to order

Tests to consider

Treatment algorithm

acute episode

coexisting inflammatory diseases (e.g., NMOSD, MOGAD, SLE or sarcoidosis)

Contributors

Authors

Cris S. Constantinescu, MD, PhD

Disclosures

Thomas M. Bosley, MD

Disclosures

Peer reviewers

Gus Gazzard, MA, MBBChir, MD, FRCOphth

Disclosures

John Selhorst, MD

Disclosures

Efstratios Mendrinos, MD

Disclosures

Jonathan Smith, MD

Disclosures

Peter Sguigna, MD

Disclosures

Differentials

Guidelines

Patient information

Log in or subscribe to access all of BMJ Best Practice

Log in or subscribe to access all of BMJ Best Practice

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