Last reviewed: 6 Nov 2021
Last updated: 18 Nov 2021

Summary

Definition

History and exam

Key diagnostic factors

  • peri-orbital/retro-ocular pain
  • loss of visual acuity with scotoma
  • colour desaturation/loss of colour vision
  • relative afferent papillary defect (RAPD)

Other diagnostic factors

  • optic disc swelling
  • neurological abnormalities of multiple sclerosis
  • phosphenes
  • Uhthoff's phenomenon
  • Pulfrich's phenomenon
  • peri-venous 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
  • FBC
  • ESR
  • C-reactive protein
  • VDRL
  • neuromyelitis optica (NMO) antibody (aquaporin-4, AQP4 antibody) and anti-myelin oligodendrocyte glycoprotein (MOG) antibody
  • uric acid
  • serum ACE
  • ANA

Investigations to consider

  • Lyme titre
  • B12 and folate
  • cerebrospinal fluid analysis

Treatment algorithm

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
Thomas M. Bosley

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.

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