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Neuro-oncology

An Atypical Case of Optic Neuropathy

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

1 Credit CME

JAMA Ophthalmology

Published Online: November 18, 2021

JAMA Ophthalmology Clinical Challenge

Article Information and Disclosures

Katherine J. Williams, MD¹;

Richard C. Allen, MD, PhD^1,2

Author Affiliations

¹Department of Ophthalmology, Baylor College of Medicine/Cullen Eye Institute, Houston, Texas
²Section of Ophthalmology, Department of Head and Neck Surgery, MD Anderson Cancer Center, Houston, Texas

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A 17-year-old previously healthy girl presented with 3 months of decreased vision in the right eye associated with throbbing pain with eye movement. The patient had no history of weakness, incontinence, or severe headaches. Initial presentation was notable for hand motion visual acuity with a 2+ relative afferent pupillary defect on the right; fundus examination revealed grade IV disc edema with obscuration of all major vessels on the disc (Figure, A). A magnetic resonance image (MRI) of the brain with and without gadolinium contrast was degraded by motion and orthodontic artifact but was suggestive of optic nerve enhancement consistent with optic neuritis. Results of serology tests and cerebral spinal fluid analysis were negative for infectious and inflammatory etiologies, including syphilis, sarcoidosis, Lyme disease, viral etiologies, multiple sclerosis, and lupus.

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A 17-year-old previously healthy girl presented with 3 months of decreased vision in the right eye associated with throbbing pain with eye movement. The patient had no history of weakness, incontinence, or severe headaches. Initial presentation was notable for hand motion visual acuity with a 2+ relative afferent pupillary defect on the right; fundus examination revealed grade IV disc edema with obscuration of all major vessels on the disc (Figure, A). A magnetic resonance image (MRI) of the brain with and without gadolinium contrast was degraded by motion and orthodontic artifact but was suggestive of optic nerve enhancement consistent with optic neuritis. Results of serology tests and cerebral spinal fluid analysis were negative for infectious and inflammatory etiologies, including syphilis, sarcoidosis, Lyme disease, viral etiologies, multiple sclerosis, and lupus.

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Article Information

Corresponding Author: Katherine J. Williams, MD, Department of Ophthalmology, Baylor College of Medicine/Cullen Eye Institute, 1977 Butler Blvd, 2nd Floor, Houston, TX 70030 (katherine.williams@bcm.edu).

Published Online: November 18, 2021. doi:10.1001/jamaophthalmol.2021.1466

Conflict of Interest Disclosures: Dr Allen has received personal fees from Horizon Pharma. No other disclosures were reported.

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

References

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AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 credit toward the CME of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.