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Key Features

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

  • 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

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

  • 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 and amiloride 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

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 ...

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