Summary
Definition
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)
Other diagnostic factors
- optic disk swelling
- neurologic abnormalities of multiple sclerosis
- Uhthoff phenomenon
- phosphenes
- Pulfrich phenomenon
- perivenous sheathing
Risk factors
- age 30 to 50 years
- female sex
- white ethnicity
- HLA-DRB1*1501 genotype
- EBV infection later in childhood
- childhood in higher latitudes
- presence of autoimmune disease
- exposure to infectious diseases, such as Lyme disease and syphilis
Diagnostic investigations
1st investigations to order
- MRI of the optic nerves
- CBC
- ESR
- C-reactive protein
- venereal disease research laboratory (VDRL)
- neuromyelitis optica (NMO) antibody (aquaporin-4, AQP4 antibody) and antimyelin oligodendrocyte glycoprotein (MOG) antibody
- uric acid
- serum ACE
- ANA
Investigations to consider
- Lyme titer
- B12 and folate
- cerebrospinal fluid analysis
Treatment algorithm
acute episode
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
UK
Attending Neurologist
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
MD
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
UK
Disclosures
GG declares that he has no competing interests.
John Selhorst, MD
Professor
Department of Neurology and Psychiatry
Saint Louis University
St. Louis
MO
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
UK
Disclosures
JS declares that he has no competing interests.
Differentials
- Lyme disease
- Syphilis
- HIV infection
More DifferentialsGuidelines
- Guidelines on diagnosis and management of neuromyelitis optica
More GuidelinesPatient leaflets
Lyme disease
Multiple sclerosis
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