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Optic Neuritis and Risk of MS: Differential Diagnosis and Management

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Optic Neuritis and Risk of MS: Differential Diagnosis and Management REVIEW CME EDUCATIONAL OBJECTIVE: Readers will be familiar with the most common causes of optic neuropathy CREDIT BENJAMIN J. OSBORNE, MD NICHOLAS J. VOLPE, MD Assistant Professor of Neurology and Niessen Professor of Ophthalmology and Ophthalmology, Departments of Neurology Neurology, Vice Chair for Clinical Practice and Ophthalmology, Georgetown University and Residency Program Director, Division Medical Center, Washington, DC of Neuro-Ophthalmology, University of Pennsylvania School of Medicine, Scheie Eye Institute, Philadelphia, PA Optic neuritis and risk of MS: Differential diagnosis and management ■■ ABSTRACT wo days ago, a 27-year-old woman no- T ticed that her vision was blurry in her Optic neuritis, a cause of sudden vision loss, often her- right eye. She has come to see her primary alds the onset of multiple sclerosis (MS) within the next care physician for advice. This is the first time few years. It is important to distinguish optic neuritis this has happened to her. She describes seeing from other types of optic neuropathy so that treatment a grayish blur over the center of her vision, but can be started promptly, possibly delaying the onset of she has not noted any other symptoms except for some soreness around the right eye, which MS. is worse with eye movements. ■■ KEY POINTS How should she be assessed and treated? Optic neuritis is most common in women in their 20s ■ IMPORTANT TO RECOGNIZE and 30s, whereas ischemic optic neuropathy, which is more common, primarily affects older people. Sudden vision loss is one of the more common problems encountered in ophthalmology and The diagnosis of optic neuritis is primarily clinical, but neurology. magnetic resonance imaging confirms the diagnosis and, Optic neuritis, a demyelinating inflamma- more importantly, assesses the risk of MS. tory condition that causes acute vision loss, is associated with multiple sclerosis (MS), and recognizing its classic clinical manifestations Intravenous methylprednisolone (Solu-Medrol) does not early is important so that appropriate diag- affect the long-term visual outcome, but it speeds visual nostic testing (magnetic resonance imaging recovery and reduces the risk of MS. Surprisingly, oral [MRI]) and treatment (corticosteroids and im- prednisone seems to increase the risk of recurrent optic munomodulators) can be started. neuritis and is therefore contraindicated. Although a comprehensive review of all the optic neuropathies is beyond the scope of Early treatment with interferon beta reduces the risk of this paper, in the pages that follow we review some of the most common causes, which may MS and should be considered in patients at high risk. be first seen by the general internist. ■ FOUR SUBTYPES OF OPTIC NEURITIS There are four subtypes of optic neuritis: • Retrobulbar neuritis (FIGURE 1), or inflamma- tion of the optic nerve behind the eye, is the form most commonly associated with MS. • Papillitis (FIGURE 2), or inflammation of the optic disc, can also be associated with MS. doi:10.3949/ccjm.76a.07268 • Perineuritis is inflammation of the optic CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 76 • NUMBER 3 MARCH 2009 181 Downloaded from www.ccjm.org on September 26, 2021. For personal use only. All other uses require permission. OPTIC NEURITIS Retrobulbar optic neuritis Papillitis FIGURE 1. In retrobulbar optic neuritis, the in- FIGURE 2. In papillitis, mild swelling and flammation and demyelination occur behind elevation of the optic disc can be seen. the globe of the eye. The optic disc appears The small splinter hemorrhage seen at 10 normal with no signs of swelling or pallor. o’clock is not typical of optic neuritis associated with multiple sclerosis. nerve sheath, sparing the optic nerve itself. ■ FEATURES: VISION LOSS AND EYE PAIN Usually, patients are older, and vision loss is mild to moderate. Perineuritis is com- Most of our current knowledge of the clinical monly due to infectious or inflammatory features of optic neuritis comes from the Op- conditions, eg, syphilis or sarcoidosis. tic Neuritis Treatment Trial (ONTT),10 con- Optic neuritis • Neuroretinitis may occur at any age. There ducted in the 1990s. This trial enrolled 457 is important to is concomitant swelling of the optic nerve patients 18 to 46 years old who had acute uni- and macula. Exudates that form around the lateral optic neuritis. The patients had to have recognize so macula give the appearance of a star. symptoms consistent with acute unilateral op- that therapy Perineuritis and neuroretinitis are not as- tic neuritis for 8 days or less. They could not can be started sociated with MS, and if they are found they have evidence of any systemic disease (except suggest another etiology. In the rest of this for MS) or have received prior treatment for early review, “optic neuritis” means retrobulbar op- MS. Therefore, this study was quite repre- tic neuritis, the form most commonly seen in sentative of the patients with optic neuritis clinical practice. that one might encounter in the clinic and is highly important in both characterizing optic ■ MOST COMMON IN YOUNG WOMEN neuritis and defining its treatment. The study found that the two most com- Acute demyelinating optic neuritis most often mon symptoms are vision loss and eye pain. affects women in their 20s and 30s.1–3 Studies Vision loss in optic neuritis typically occurs in the United States have estimated its annual over several hours to days, and vision reaches incidence to be 5.1 to 6.4 per 100,000.4,5 The a nadir within 1 to 2 weeks. Typically, patients incidence is higher in populations at higher begin to recover 2 to 4 weeks after the onset of latitudes and lower near the equator. It is less the vision loss. The optic nerve may take up to common in blacks than in whites.6 6 to 12 months to heal completely, but most pa- In children, optic neuritis is not as strongly tients recover as much vision as they are going associated with MS, especially when there is op- to within the first few months.11 More than two- tic disc swelling or bilateral involvement. Most thirds of patients have at least 20/20 vision once children have a good visual outcome, although they have fully recovered from the optic neuritis. approximately 20% may be visually disabled.7–9 Only 3% of patients become completely blind. 182 CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 76 • NUMBER 3 MARCH 2009 Downloaded from www.ccjm.org on September 26, 2021. For personal use only. All other uses require permission. OSBORNE AND VOLPE TABLE 1 Common characteristics of optic neuropathies in adults OPTIC NEURITIS ISCHEMIC OPTIC NEUROPATHY Typical age 20–40 years 50 years and older Onset Acute to subacute Acute Disc swelling Less common; if present, it is mild Very common Pain Worse on eye movement Less common Recovery at 1 year Most recover 20/20 vision Poor Associated disease Multiple sclerosis Giant cell arteritis Eye pain is very common in optic neuri- A relative afferent pupillary defect should tis (it affected 87% of patients in the ONTT) be seen in the involved eye in all patients with and typically worsens with eye movement. optic neuritis if the other eye is uninvolved The eye is also sore to touch. The pain gener- and healthy.12 The best way to elicit this sign ally begins at the same time as the visual loss is to perform the swinging light test in a dark and improves along with it. Eye movements room with the patient fixating at a distant also may bring about photopsia (flickering or target, which prevents miosis due to accom- flashes of light), a symptom reported by 30% modation. When the light is swung to the in- of the ONTT participants. volved eye, the pupil dilates because less light Loss of color vision out of proportion to stimulus reaches the midbrain through the af- the loss of visual acuity is characteristic of op- fected optic nerve. As the optic nerve heals tic neuropathies. In the ONTT, 88% of the and recovers, this sign may become subtle, but involved eyes had abnormal color vision as it persists in more than 90% of cases.12 Ischemic optic assessed by the Ishihara test (using pseudo- neuropathy is isochromatic plates), and 94% as assessed by Findings on funduscopy the Farnsworth-Munsell 100 hue test, which Examination of the fundus is helpful in the more common is more sensitive but cumbersome. The most clinical diagnosis of optic neuritis. after age 50 common patterns of color vision loss in optic Two-thirds of the ONTT patients had nerve disease are loss of red (protanopia) and retrobulbar neuritis, and one-third had papil- green (deutranopia). litis. If optic nerve swelling is present, it is A good way to screen for loss of color vi- typically mild. sion is to test for color desaturation. First, ask Peripapillary hemorrhages were exceed- the patient to fixate with the normal eye on a ingly rare in the cases of papillitis (only 6%) bright red object (for example, the cap from a and were associated with a very low to zero bottle of ophthalmic mydriatic drops or a pen risk of developing MS. If peripapillary hemor- cap). Then ask the patient to compare the in- rhages are found on examination, one should tensity of the redness between the good eye consider another diagnosis, such as anterior and the affected eye. The patient can quantify ischemic optic neuropathy.11 the color desaturation by rating what percent- age of red is lost in the affected eye compared ■ CASE CONTINUED with the normal eye. Temporary exacerbations of visual prob- Our patient undergoes a neurologic examination, lems during fever (the Uhthoff phenomenon) which reveals an afferent pupillary defect in the can occur in patients who have had optic neuri- right eye and visual acuity of 20/100 in the right tis.
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