Adult Optic Neuritis Clinical Presentation

Updated: Jan 22, 2021
  • Author: Andrew A Dahl, MD, FACS; Chief Editor: Edsel B Ing, MD, MPH, FRCSC, PhD, MA  more...
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Presentation

History

A history of preceding viral illness and pain on eye movement may be present. Typically, patients with first-time acute optic neuritis (ON) are otherwise healthy young adults. Patients with ON experience rapidly developing impairment of vision in 1 eye or, far less commonly, both eyes during an acute attack. [3]

Symptoms of dyschromatopsia (change in color perception) in the affected eye may occasionally be more prominent than the decreased vision. [4]  In nearly all patients with ON, the visual changes are associated with retro-orbital or ocular pain, usually exacerbated by eye movement. The pain may precede the visual loss.

Patients may report vision loss exacerbated by heat or exercise (Uhthoff phenomenon). Objects moving in a straight line may appear to have a curved trajectory (Pulfrich effect) when viewed bilaterally, presumably as a result of asymmetric conduction between the optic nerves.

Patients with MS may have recurrent attacks of ON [5] ; therefore, a history of episodes of decreased vision in the same or the fellow eye may be elicited. A history of neurologic problems, such as transient episodes of extremity or facial numbness, weakness, or balance difficulties, suggests a diagnosis of MS; a family history of MS may exist.

Neuromyelitis optica is characterized by ON and myelitis in a close temporal relationship [6, 7, 8, 9] ; however, ON can occasionally precede the myelopathy. Some patients with neuromyelitis optica (NMO) have relapses limited to the optic nerves and spinal cord.

In male patients with bilateral, sequential optic neuropathy with little recovery of vision, Leber hereditary optic neuropathy, rather than demyelinating optic neuritis, should be considered as the diagnosis. Patients with Leber hereditary optic neuropathy may have a history of vision loss in maternal uncles.

Chronic relapsing inflammatory optic neuropathy (CRION) is a form of inflammatory optic neuropathy that is frequently bilateral and often painful. It is characterized by relapses and remissions. Magnetic resonance imaging (MRI) findings in the brains of patients with CRION are normal, and MRI of the optic nerves often, but not always, shows high-signal abnormalities that enhance. Corticosteroid treatment is effective in reducing symptoms of CRION, although long‐term immunosuppression is often necessary. The syndrome behaves in a way that is typical of granulomatous optic neuropathy, but during long-term follow-up, no systemic sarcoidosis has been implicated. [41]  

 

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Physical Examination

In a patient with a typical initial acute case of optic neuritis (ON), findings on a general physical examination are normal. Pupillary light reaction is decreased in the affected eye, and a relative afferent pupillary defect (RAPD) or Marcus Gunn pupil is commonly found. In bilateral cases, the RAPD may not be apparent.

Measurement of visual acuity reveals variation in reduction from no reduction to complete visual loss. Patients with ON will all have at least mild subjective decreases in color vision and perception of brightness in the affected eye. All patients with decreased visual acuity also have abnormal contrast sensitivity and color vision, as revealed by examination using a Pelli-Robson chart and Ishihara color plates, respectively.

A central scotoma is most commonly seen in patients with ON; however, results of the Optic Neuritis Treatment Trial (ONTT) suggest that altitudinal field defects, arcuate defects, and nasal steps are more common than central scotomas and centrocecal scotomas. Visual field examination may show a central scotoma, often with peripheral extension in any direction. Generalized depression of the entire visual field in the affected eye may be encountered.

In two thirds of cases of acute optic neuritis, the optic nerve appears normal because of only retrobulbar involvement of the nerve. One third of patients with ON have a swollen disc (papillitis). With time, the optic nerve may become pale.

The disc edema of ON, when present, is often diffuse. The presence of segmental changes, altitudinal swelling, pallor, arterial attenuation, and splinter hemorrhages suggest other diagnoses (eg, anterior ischemic optic neuropathy). [42]

Findings on retinal examination are usually normal. If a dilated fundus examination is not performed, other conditions such as central serous retinopathy and retinal detachment may be mistaken for ON. [43]

Patients with NMO often develop a severe, bilateral form of ON and myelitis. Bitemporal or junctional visual field defects, indicating chiasm involvement, may be present. Myelitis may be associated with localized back or radicular pain and the Lhermitte sign (spine or limb paresthesias elicited by neck flexion) early in the course of the disease. Severe neurologic deficits, including paraplegia, are typical. Symptoms such as respiratory failure or hiccups may occur when the cervical spinal cord lesions extend into the medulla.

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