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Bilateral Disc Edema Masquerade

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

A 58-year-old man with a history of hypertensive retinopathy presented with acute-onset, painless vision loss in his right eye accompanied by tinnitus and severe intermittent headaches. His extraocular movements were full, and he had no other neurologic deficits. His visual acuity was 20/400 OD and 20/30 OS, respectively, and his fundi were notable for bilateral optic nerve edema. Results of recent computed tomography and angiography of the head and neck and magnetic resonance imaging of the brain and orbits were reportedly normal, and lumbar puncture revealed an opening pressure of 26 cm H2O (normal range, 6 to 25 cm H2O) with elevated protein. The patient was given acetazolamide and referred to neuro-ophthalmology.

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Primary angiitis of the central nervous system

B. Repeated lumbar puncture with serologies

Bilateral disc edema, transient vision loss, and elevated opening pressure on lumbar puncture are suggestive of intracranial hypertension, which may be idiopathic intracranial hypertension or secondary to a mass-occupying lesion, dural venous sinus thrombosis, or another underlying disease, including anemia, Addison disease, or systemic lupus erythematosus.1 While tighter blood pressure control (choice A) would be helpful in hypertensive retinopathy, elevated blood pressure medications do not mitigate elevated intracranial pressure. With negative laboratory findings for autoimmune or inflammatory disease, there is no indication to start high-dose corticosteroids (choice C). The patient had persistent visual field deficits despite acetazolamide use, which requires further investigation beyond monitoring with serial testing (choice D). A repeated lumbar puncture (choice B) revealed an opening pressure of 18 cm H2O, an IgG level of 6.6 mg/dL (normal range, 0.5 to 5.9 mg/dL; to convert to grams per liter, multiply by 0.01), and 4 oligoclonal bands (normal range, 0 to 1). Throughout this workup, the patient began to develop new neurological symptoms, including persistent left cranial nerve V numbness, paresthesia, fatigue, cold intolerance, throbbing headache, and bradyphrenia (ie, slowing of cognition).

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Article Information

Corresponding Author: Steven Carter, MD, Gavin Herbert Eye Institute, Department of Ophthalmology, University of California, Irvine, School of Medicine, 850 Health Sciences Rd, Irvine, CA 92697 (steven.carter@uci.edu).

Published Online: August 5, 2021. doi:10.1001/jamaophthalmol.2021.0096

Conflict of Interest Disclosures: The authors acknowledge departmental support from an unrestricted Research to Prevent Blindness grant.

Additional Contributions: We thank the patient for granting permission to publish this information. We thank Mari Perez-Rosendahl, MD, University of California, Irvine, Department of Pathology, for her expertise in the histopathological analysis of the brain biopsy specimen.

References
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Madriz Peralta  G , Cestari  DM .  An update of idiopathic intracranial hypertension.   Curr Opin Ophthalmol. 2018;29(6):495-502. doi:10.1097/ICU.0000000000000518PubMedGoogle ScholarCrossref
2.
Birnbaum  J , Hellmann  DB .  Primary angiitis of the central nervous system.   Arch Neurol. 2009;66(6):704-709. doi:10.1001/archneurol.2009.76PubMedGoogle ScholarCrossref
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6.
Salvarani  C , Brown  RD  Jr , Calamia  KT ,  et al.  Angiography-negative primary central nervous system vasculitis.   Medicine (Baltimore). 2008;87(5):264-271. doi:10.1097/MD.0b013e31818896e1PubMedGoogle ScholarCrossref
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Beuker  C , Schmidt  A , Strunk  D ,  et al.  Primary angiitis of the central nervous system.   Ther Adv Neurol Disord. Published online July 9, 2018 doi:10.1177/1756286418785071Google Scholar
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