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 DR(2)15 genetic mutation
- 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)
- uric acid
- serum ACE
- ANA
Investigations to consider
- Lyme titer
- B12 and folate
- cerebrospinal fluid analysis
- neuromyelitis optica (NMO) antibody (aquaporin-4, AQP4 antibody) and anti-myelin oligodendrocyte glycoprotein (MOG) antibody
Treatment algorithm
Contributors
Authors
Chair of Neurology
School of Medical and Surgical Sciences
Division of Stroke Medicine
University of Nottingham
Nottingham
UK
Disclosures
CSC has received research support, support for travel to scientific meetings, or consultancy fees from Biogen, Bayer, Genzyme, Merck, Morphosys, Novartis, Roche, Sanofi-Pasteur, MSD, and Teva. CSC is an author of a number of references cited in this topic.

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
Consultant Ophthalmic Surgeon
King's College Hospital
Honorary Research Fellow
Institute of Ophthalmology
London
UK
Disclosures
GG declares that he has no competing interests.
Professor
Department of Neurology and Psychiatry
Saint Louis University
St. Louis
MO
Disclosures
JS declares that he has no competing interests.
Department of Ophthalmology
Geneva University Hospitals
Geneva
Switzerland
Disclosures
EM declares that he has no competing interests.
Specialist Registrar in Ophthalmology
Royal Victoria Infirmary
Newcastle
UK
Disclosures
JS declares that he has no competing interests.
Use of this content is subject to our disclaimer