Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 7-22: Optic Neuritis + Key Features Download Section PDF Listen +++ +++ Essentials of Diagnosis ++ Subacute usually unilateral visual loss Pain exacerbated by eye movements Optic disk usually normal in acute stage but subsequently develops pallor +++ General Considerations ++ Strongly associated with demyelinating disease, particularly multiple sclerosis but also acute disseminated encephalomyelitis Also occurs In sarcoidosis In neuromyelitis optica spectrum disorder In association with serum antibodies to myelin oligodendrocyte glycoprotein In children following viral infections In varicella zoster virus infection In various autoimmune disorders, particularly systemic lupus erythematosus and Sjögren syndrome In persons receiving biologics therapy (eg, anti-TNF agents and natalizumab) In spread of inflammation from the meninges, orbital tissues, or paranasal sinuses + Clinical Findings Download Section PDF Listen +++ ++ Unilateral loss of vision that usually develops over a few days Vision ranges from 20/30 (6/9) to no light perception with more severe visual loss being associated with low serum vitamin D levels Pain behind the eye, exacerbated by eye movements Field loss is usually a central scotoma, but a wide range of monocular field defects is possible Particular loss of color vision A relative afferent pupillary defect In about two-thirds of cases, the optic nerve is normal during the acute stage (retrobulbar optic neuritis) In one-third of cases, the optic disk is swollen (papillitis) with occasional flame-shaped peripapillary hemorrhages Visual acuity usually improves within 2–3 weeks and returns to 20/40 (6/12) or better in 95% of previously unaffected eyes Optic atrophy develops subsequently if there has been damage to sufficient optic nerve fibers + Diagnosis Download Section PDF Listen +++ +++ Imaging Studies ++ MRI of the head and orbits should be obtained to exclude a lesion compressing the optic nerve and to identify atypical optic neuritis In patients in whom visual recovery does not occur In patients with other atypical features, including continuing deterioration of vision or persisting pain after 2 weeks + Treatment Download Section PDF Listen +++ ++ Intravenous methylprednisolone (1 g daily for 3 days followed by a tapering course of oral prednisolone) Has been shown to accelerate visual recovery, although in clinical practice, the oral taper is not often prescribed and oral methylprednisolone may be used Use in an individual patient is determined by the degree of visual loss, the state of the fellow eye, and the patient's visual requirements Phenytoin may be neuroprotective in typical optic neuritis Atypical optic neuritis due to sarcoidosis, neuromyelitis optica, herpes zoster, or systemic lupus erythematosus Requires more immediate and prolonged corticosteroid therapy May require plasma exchange May require long-term immunosuppression + Outcome Download Section PDF Listen +++ +++ Prognosis ++ Multiple sclerosis will develop in 50% of patients with clinically isolated optic neuritis The visual and neurologic prognosis is good The major risk factors are female sex and multiple white matter lesions on brain MRI Many disease-modifying drugs are available to reduce the risk of further neurologic episodes and potentially the accumulation of disability, but each has adverse effects that in some instances are life-threatening Fingolimod is associated with macular edema Retinal nerve fiber layer optical coherence tomography quantifies axonal damage that can be used to monitor disease progression Optic neuritis due to sarcoidosis, neuromyelitis optica, herpes zoster, or systemic lupus erythematosus generally has a poorer prognosis +++ When to Refer ++ All patients should be referred urgently for ophthalmologic or neurologic assessment + References Download Section PDF Listen +++ + +Burton JM et al. A prospective cohort study of vitamin D in optic neuritis recovery. Mult Scler. 2017 Jan;23(1):82–93. [PubMed: 27037181] + +de Andrade FA et al. Neuro-ophthalmologic manifestations in systemic lupus erythematosus. Lupus. 2017 Apr;26(5):522–8. [PubMed: 28394224] + +Deschamps R et al. Etiologies of acute demyelinating optic neuritis: an observational study of 110 patients. Eur J Neurol. 2017 Jun;24(6):875–9. [PubMed: 28477397] + +Jenkins TM et al. Optic neuritis: the eye as a window to the brain. Curr Opin Neurol. 2017 Feb;30(1):61–66. [PubMed: 27906756] + +Kale N. Optic neuritis as an early sign of multiple sclerosis. Eye Brain. 2016 Oct 26;8:195–202. [PubMed: 28539814] + +Morrow MJ et al. Should oral corticosteroids be used to treat demyelinating optic neuritis? J Neuroophthalmol. 2017 Dec;37(4):444–50. [PubMed: 28857910] + +Osinga E et al. Time is vision in recurrent optic neuritis. Brain Res. 2017 Oct 15;1673:95–101. [PubMed: 28823952] + +Patterson SL et al. Neuromyelitis optica. Rheum Dis Clin North Am. 2017 Nov;43(4):579–91. [PubMed: 29061244] + +Raftopoulos R et al. Phenytoin for neuroprotection in patients with acute optic neuritis: a randomised, placebo-controlled, phase 2 trial. Lancet Neurol. 2016 Mar;15(3):259–69. [PubMed: 26822749] + +Zhao G. Clinical characteristics of myelin oligodendrocyte glycoprotein seropositive optic neuritis: a cohort study in Shanghai, China. J Neurol. 2018 Jan;265(1):33–40. [PubMed: 29101456]