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A 2-Year History of Diplopia, Optic Disc Edema, and Amaurosis

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A right-handed man in his 50s with a medical history of hypertension was referred for optic disc edema in the right eye and double vision. He was initially seen locally for new-onset double vision in upgaze without other visual symptoms. The patient had a 3–prism diopter left hypertropia in upgaze with full motility and was incidentally found to have optic disc edema in the right eye. His best-corrected visual acuity was 20/15 OU. Automated visual fields were normal, and there was no relative afferent pupillary defect. Magnetic resonance imaging (MRI) revealed no apparent abnormalities (Figure 1A). During the subsequent 6 months, the patient experienced graying of vision on rightward gaze. Results of an evaluation again demonstrated normal visual acuity and visual fields. Results of a local workup were negative, including acetylcholine receptor antibody. The patient presented to our clinic 2 years after initial symptom onset with continued graying of vision on eccentric gaze. His best-corrected visual acuity on examination was 20/25 OD and 20/20 OS. Relative afferent pupillary defect was noted in the right eye. There was prominent chronic-appearing optic disc edema in the right eye without abnormalities in the left eye (Figure 1B). Results of visual field testing showed superior greater than inferior peripheral field loss in the right eye and normal visual field in the left eye.

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Optic nerve sheath meningioma

C. Another MRI scan of the orbit

The initial presentation of unilateral optic disc edema with intact vision raises an interesting differential diagnosis, including incipient nonarteritic anterior ischemic optic neuropathy (NAION), unilateral papilledema, compressive optic neuropathy, and optic perineuritis. Risk factor modification (choice A) would not be correct because it is the next step in the management of NAION, for which hypertension, hyperlipidemia, diabetes, and obstructive sleep apnea are commonly found modifiable vascular risk factors.1 Visual acuity can be normal in the setting of disc edema in incipient NAION; however, the duration of optic swelling would have been shorter, and NAION would not have caused gaze-evoked amaurosis.1,2 Nonarteritic anterior ischemic optic neuropathy most commonly causes an inferior altitudinal defect, whereas our patient had peripheral field loss in the right eye. A lumbar puncture (choice B) is not the next step, but it would be used to obtain the opening pressure to diagnose papilledema, which can also cause visually asymptomatic disc edema. However, papilledema causes transient visual obscurations and not gaze-evoked amaurosis.

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Article Information

Corresponding Author: Jacqueline A. Leavitt, MD, Department of Ophthalmology, Mayo Clinic, 200 First St SW, Rochester, MN 55905 (leavitt.jacqueline@mayo.edu).

Published Online: November 1, 2018. doi:10.1001/jamaophthalmol.2018.2604

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and no disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Miller  NR, Arnold  AC.  Current concepts in the diagnosis, pathogenesis and management of nonarteritic anterior ischaemic optic neuropathy.  Eye (Lond). 2015;29(1):65-79. doi:10.1038/eye.2014.144PubMedGoogle ScholarCrossref
2.
Hayreh  SS, Zimmerman  MB.  Incipient nonarteritic anterior ischemic optic neuropathy.  Ophthalmology. 2007;114(9):1763-1772. doi:10.1016/j.ophtha.2006.11.035PubMedGoogle ScholarCrossref
3.
Bradbury  PG, Levy  IS, McDonald  WI.  Transient uniocular visual loss on deviation of the eye in association with intraorbital tumours.  J Neurol Neurosurg Psychiatry. 1987;50(5):615-619. doi:10.1136/jnnp.50.5.615PubMedGoogle ScholarCrossref
4.
Dutton  JJ, Anderson  RL.  Idiopathic inflammatory perioptic neuritis simulating optic nerve sheath meningioma.  Am J Ophthalmol. 1985;100(3):424-430. doi:10.1016/0002-9394(85)90506-9PubMedGoogle ScholarCrossref
5.
Purvin  V, Kawasaki  A, Jacobson  DM.  Optic perineuritis: clinical and radiographic features.  Arch Ophthalmol. 2001;119(9):1299-1306. doi:10.1001/archopht.119.9.1299PubMedGoogle ScholarCrossref
6.
Turbin  RE, Pokorny  K.  Diagnosis and treatment of orbital optic nerve sheath meningioma.  Cancer Control. 2004;11(5):334-341. doi:10.1177/107327480401100508PubMedGoogle ScholarCrossref
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