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.
Optic neuritis (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 multiple sclerosis (MS). 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 to 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)
Other diagnostic factors
- optic disk swelling
- neurologic abnormalities of multiple sclerosis
- Uhthoff phenomenon
- Pulfrich phenomenon
- perivenous sheathing
- 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
1st investigations to order
- MRI of the optic nerves
- 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
Investigations to consider
- Lyme titer
- B12 and folate
- cerebrospinal fluid analysis
coexisting inflammatory diseases (e.g., NMOSD, MOGAD, SLE or sarcoidosis)
- Lyme disease
- HIV infection
- Guidelines on diagnosis and management of neuromyelitis optica
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