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Quiz

Neuro-ophthalmology

Diplopia and Ptosis in an Older Woman

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

1 Credit CME

JAMA Neurology

Published Online: July 25, 2022

JAMA Neurology Clinical Challenge

Article Information and Disclosures

Jeannette Y. Stallworth, MD¹;

J. Palmer Greene, MD²;

Nailyn Rasool, MD¹

Author Affiliations

¹Department of Ophthalmology, University of California, San Francisco, San Francisco
²Department of Neurology, University of California, San Francisco, San Francisco

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A 75-year-old woman presented with horizontal binocular diplopia, right-sided ptosis, and a new headache that was progressive over 3 days. She reported difficulty opening her jaw, pain when chewing, and a 2.3-kg weight loss. The week prior, she experienced left-sided ptosis that persisted for 2 days and subsequently resolved. She denied vision changes, eye pain, scalp tenderness, and myalgias. Past medical history included schizophrenia, hypothyroidism, and supraventricular tachycardia.

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A. Giant cell arteritis

Facial and extraocular muscles are typically spared by the myositis associated with aminoacyl transfer RNA synthetase antibodies, and the lack of other features of antisynthetase syndrome (interstitial lung disease, nonerosive arthritis, Raynaud phenomenon) makes this diagnosis less likely. Immunoglobulin G4–related disease can cause enlargement of the extraocular muscles, creating a restrictive pattern of motility but may also be accompanied by hypertrophic pachymeningitis and sclerosing lesions of the abdomen and lungs. Lastly, although the history of transient contralateral ptosis may be reminiscent of myasthenia gravis, the lack of fatiguability, normal ice-pack test results, and soft tissue changes on magnetic resonance imaging suggest an alternate etiology.

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Article Information

Corresponding Author: Jeannette Y. Stallworth, MD, Department of Ophthalmology, University of California, San Francisco, 490 Illinois St, 5th Floor, Room 5X8, San Francisco, CA 94143 (jeannette.stallworth@ucsf.edu).

Published Online: July 25, 2022. doi:10.1001/jamaneurol.2022.1982

Conflict of Interest Disclosures: None reported.

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

References

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González-Gay MA , García-Porrúa C , Llorca J , et al. Visual manifestations of giant cell arteritis—trends and clinical spectrum in 161 patients. Medicine (Baltimore). 2000;79(5):283-292. doi:10.1097/00005792-200009000-00001 PubMed Google Scholar Crossref

Dimant J , Grob D , Brunner NG . Ophthalmoplegia, ptosis, and miosis in temporal arteritis. Neurology. 1980;30(10):1054-1058. doi:10.1212/WNL.30.10.1054 PubMed Google Scholar Crossref

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Arunagiri G , Santhi S , Harrington T . Horner syndrome and ipsilateral abduction deficit attributed to giant cell arteritis. J Neuroophthalmol. 2006;26(3):231-232. doi:10.1097/01.wno.0000235562.42894.33 PubMed Google Scholar Crossref

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 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

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