A 30-Year-Old Woman with Vision Loss and Painful Eye Movements

CMAJ Practice CME

What is your call? A 30-year-old woman with vision loss and painful eye movements

Jonathan A. Micieli MD CM, Edward Margolin MD

30-year-old woman presented to the demyelination.) Laboratory and imaging studies Competing interests: None emergency department with a two-day may be used for prognostic purposes, but they declared. A history of pain in her right eye that was are not required. As was the case with this This article has been peer worse with eye movements. She also complained patient, the typical person with optic neuritis is reviewed. of blurred vision in the right eye. She had no float- young (mean age 32 yr), female (3:1 ratio of Correspondence to: ers or eye redness and did not have recent trauma. women to men) and white (> 75% of patients).1 Jonathan Micieli, jonathan Her ocular and medical history were unremark- Patients present with subacute loss of vision that [email protected] able, and she was not taking medications. develops over hours or days. In most instances, the CMAJ 2015. DOI:10.1503 On physical examination, the patient appeared vision loss is associated with orbital pain (in 92%) /cmaj.141389 well and had normal vital signs. Her best cor- that is worse with eye movements (in 87%).1 The rected visual acuity was 20/50 in the right eye pain is thought to be due to mechanical irritation of and 20/20 in the left eye, and the intraocular pres- the inflamed optic nerve.2 About one-third of sures were normal at 13 mm Hg. The patient had patients also note photopsia.1 a relative afferent pupillary defect in her right eye, An important finding on physical examination and her extraocular movements were full. There is a relative afferent pupillary defect, which is was no proptosis or periorbital swelling. Findings always seen in optic neuritis except in the rare on slit-lamp examination were normal, and fundu- instance of symmetric bilateral disease. Other scopic examination with pupil dilation showed a important findings on examination are decreased normal macula, peripheral retina and optic nerve. central visual acuity (although all ranges of vision Results of a full neurologic examination were oth- from 20/15 to no light perception may be present) erwise normal. and edema of the optic nerve head (in one-third of patients), which was not seen in this patient.1 The clinical data for the diagnosis and treat- What is the most appropriate ment of optic neuritis are derived from a well- next step? designed randomized controlled trial: the Optic Neuritis Treatment Trial.1 The trial enrolled more a. Order complete blood count, antinuclear anti- than 400 consecutive patients aged 18–45 years body test and venereal disease research lab from multiple sites across North America who oratory test had an acute clinical syndrome consistent with b. Diagnose optic neuritis and arrange for non- unilateral optic neuritis and whose visual symp- urgent magnetic resonance imaging (MRI) of toms were present for up to eight days.1 the brain with ophthalmology follow-up Optic neuritis is diagnosed when patients who c. Obtain computed tomography of the orbits to fit the demographic pattern present with typical rule out compressive lesion of the optic nerve symptoms (i.e., acute to subacute decrease in d. Perform lumbar puncture to rule out infec- vision and eye pain that is worse with extraocular tious causes and for oligoclonal band screen- movements) and findings on physical examination ing of the cerebrospinal fluid (CSF) (i.e., relative afferent pupillary defect with or without optic nerve edema). Reduced colour vision and The answer is (b). Optic neuritis is an inflamma- visual field defects may be seen on formal testing. tory, demyelinating disease of the optic nerve Atypical cases that do not fit the usual demo- typically diagnosed on clinical grounds without graphic pattern or clinical presentation require a neuroimaging. (By convention, the term “optic more urgent referral to an ophthalmologist to neuropathy” will be used in this article when the assess for other causes of vision loss. The differen- cause of inflammation of the optic nerve is not tial diagnosis includes ischemic optic neuropathy,

infectious optic neuropathy (e.g., due to syphilis), Having optic neuritis as the first manifestation compressive lesion of the optic nerve and infiltra- of MS is considered a favourable prognostic fac- tive disease such as sarcoid or central nervous sys- tor.4 Among the patients in the Optic Neuritis tem lymphoma. Treatment Trial who subsequently received a In the Optic Neuritis Treatment Trial, all pa- diagnosis of clinically definite MS, there was a tients received the fluorescent treponemal antibody relatively low frequency of substantial disability absorption test, and none was found to have active (the Expanded Disability Status Scale score at syphilis as the cause of optic neuritis.1 Similarly, 10-year follow-up was < 3 in 65% of patients antinuclear antibody testing was noncontributory, and > 6 in 17%).5 which makes answer (a) less likely. A person with a compressive lesion of the optic nerve (c) usually How do you respond when presents with gradual vision loss and, depending on the location of the lesion, may have other asso- the patient asks if her vision will ciated signs such as proptosis or optic nerve edema return and if she needs treatment? or pallor, which was not seen in this patient. More- over, lumbar puncture was performed in 31.5% of a. Unfortunately, the vision will not recover to a the patients in the Optic Neuritis Treatment Trial great extent, and treatments are still in the and did not reveal an additional cause for the vi- experimental phase sion loss other than demyelinating disease.1 Al- b. The vision will return only if prompt treatment though CSF oligoclonal bands may be seen in with intravenous corticosteroids is started some patients with multiple sclerosis (MS), this c. The vision will improve minimally over the test (d) is not indicated for a patient with a first next six months; no treatment is needed presentation of typical optic neuritis. d. The vision will most likely return close to baseline, and treatment with intravenous How do you respond when corticosteroids will only hasten visual recovery the patient asks if she has The answer is (d). The typical course of visual multiple sclerosis? recovery begins at two weeks, peaks at one month and continues up to one year after symptom a. Multiple sclerosis eventually develops in all onset.6 Most patients (95%) experience a return of patients with optic neuritis and is most often their central visual acuity back to baseline, diagnosed after five years although many still feel that their vision is “faded” b. Optic neuritis is only rarely associated with MS or has diminished contrast.6 The course of visual c. Magnetic resonance imaging of the brain is recovery is important in confirming the diagnosis the best way to determine MS risk, and a of optic neuritis; therefore, patients should be fol- more accurate prognosis can be given after lowed at regular intervals. Patients whose vision the MRI results does not recover require further investigations for d. The diagnosis of MS is almost certain, and other causes of vision loss. disease-modifying therapy should be started The Optic Neuritis Treatment Trial found that intravenous corticosteroid treatment with methyl The answer is (c). The strongest predictor of MS prednisolone (1 g/d for 3 d) followed by oral is the presence of white matter lesions. The Op- prednisone treatment (1 mg/kg for 11 d) given tic Neuritis Treatment Trial found that, among within 8 days after onset of symptoms improved patients with at least one white matter lesion of the speed of visual recovery by about 4 to 6

at least 3 mm in diameter on T2-weighted MRI weeks but had no effect on visual outcomes at sequence, the 15-year risk of clinically definite 6 months to 10 years.7 Oral corticosteroid treat- MS was 72%.3 Among those who had no le- ment (1 mg/kg for 14 d) was found to be in sions, the 15-year risk was 25%. To meet MS effective and increased the risk of new episodes diagnostic criteria in the trial, patients had to of optic neuritis.7 have a clinical examination documenting a sec- ond new neurologic deficit attributable to de Discussion myelination that was consistent with neurologic symptoms, lasted at least 24 hours and occurred Optic neuritis is a demyelinating inflammatory at least four weeks after the initial optic neuritis condition most often seen in young white event.3 Recurrent episodes of optic neuritis in women. Optic neuritis is the presenting feature either eye were not considered in the diagnostic of MS in 15% to 20% of patients and occurs in criteria for MS, because demyelinating events 50% at some time during the course of their ill- need to be separated both in space and time. ness.8 The incidence is highest in populations

674 CMAJ, June 16, 2015, 187(9) Practice

6. Optic Neuritis Study Group. Visual function 15 years after optic located at higher latitudes, such as the northern neuritis: a final follow-up report from the optic neuritis treat- United States and Canada, and is lowest in ment trial. Ophthalmology 2008;115:1079-82.e5. regions closer to the equator.9 The pathophysiol- 7. Beck RW, Cleary PA, Anderson MM Jr, et al.; Optic Neuritis Study Group. A randomized, controlled trial of corticosteroids in the treat- ogy is similar to that of MS and is thought to be ment of acute optic neuritis. N Engl J Med 1992;326:581-8. immune-mediated. 8. Balcer LJ. Clinical practice. Optic neuritis. N Engl J Med 2006; 354:1273-80. Optic neuritis is a clinical diagnosis that does 9. Percy AK, Nobrega FT, Kurland LT. Optic neuritis and multiple not require laboratory findings or neuroimaging sclerosis: an epidemiologic study. Arch Ophthalmol 1972;87:135-9. to make a diagnosis. Patients should be followed Affiliation: Department of Ophthalmology and Vision Sci- at regular intervals to ensure that they follow the ence, University of Toronto, Toronto, Ont. typical course of visual recovery and to assess Contributors: Both authors contributed equally to the writ- for new neurologic symptoms. Patients should ing and revising of the manuscript, accepted the final version be reassured that a diagnosis of optic neuritis submitted for publication and agreed to act as guarantors of does not necessarily mean that MS will develop the work. and that if MS does develop, there is a good chance it will follow a more benign course. CMAJ invites submissions to “What is your call?” Clinical details (including images) are References presented with a multiple-choice question about 1. Optic Neuritis Study Group. The clinical profile of optic neuritis: experience of the Optic Neuritis Treatment Trial. Arch Oph- the diagnosis. The answer and a brief discussion thalmol 1991;109:1673-8. of the condition follow. We specifically invite 2. Fazzone HE, Lefton DR, Kupersmith MJ. Optic neuritis: corre- submissions illustrating common or important lation of pain and magnetic resonance imaging. Ophthalmology 2003;110:1646-9. radiographic and electrocardiographic diagnoses 3. Optic Neuritis Study Group. Multiple sclerosis risk after optic of appeal to a general audience. We require neuritis: final Optic Neuritis Treatment Trial follow-up.Arch Neurol 2008;65:727-32. authors to obtain consent from the patient for 4. Runmarker B, Andersen O. Prognostic factors in a multiple scle- publication of his or her story (form available at rosis incidence cohort with twenty-five years of follow-up. www.cmaj.ca/site/authors/checklist.xhtml). Submit Brain 1993;116:117-34. 5. Beck RW, Smith CH, Gal RL, et al.; Optic Neuritis Study manuscripts online at http://mc.manuscriptcentral Group. Neurologic impairment 10 years after optic neuritis. .com/cmaj. Arch Neurol 2004;61:1386-9.

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