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Bilateral Central Scotoma in a Middle-aged Man

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

A 43-year-old man presented as a referral for bilateral central vision loss. His medical history was significant for Crohn disease controlled with monthly injections of ustekinumab (Stelara; Janssen Biotech Inc). Symptoms of fever, headache, and myalgia began 1 week prior following a tick bite. He was prescribed oral doxycycline hyclate for suspected Lyme disease. Owing to persistent fever while receiving oral doxycycline hyclate, he was admitted for intravenous administration of doxycycline hyclate, and the fever resolved. He was found to be anemic (hemoglobin concentration, 6.7 g/dL; normal concentration, 13.5-17.5 g/dL) (to convert hemoglobin concentration to g/L, multiply by 10), requiring transfusion. While hospitalized, he developed acute bilateral central scotoma. Magnetic resonance imaging of the orbit and brain with and without contrast was unremarkable. Infectious serology test results for Lyme disease, syphilis, malaria, Anaplasma species, Cryptococcus species, Rocky Mountain spotted fever, and rickettsia were negative. Rheumatologic serology test results for antinuclear antibodies, including Sjögren anti-Ro and anti-La, anti-DNA, and anti-Smith, were also negative. Antineutrophil cytoplasmic antibody testing revealed elevated proteinase 3 antibody level. In the setting of anemia, infectious-disease consultants raised concern for babesiosis. Peripheral blood smear and polymerase chain reaction were performed; empirical therapy of azithromycin, 500 mg daily, plus atovaquone, 750 mg twice daily, was initiated. Polymerase chain reaction results for babesiosis later returned as negative.

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Acute macular neuroretinopathy

C. Order optical coherence tomography

In this middle-aged man with bilateral central scotoma following recent illness, acute macular neuroretinopathy (AMN) was suspected. Near-infrared imaging showed hyporeflectivity of the parafovea, and spectral-domain optical coherence tomography (SD-OCT) revealed subfoveal disruption of the outer retina (Figure). Fundus autofluorescence and fluorescein angiography findings were unremarkable. A diagnosis of AMN was based on the examination and imaging findings. Findings of indocyanine green angiography (choice A) are typically normal in patients with AMN, and this procedure is indicated only if other causes of posterior uveitis are suspected. Immunomodulating agents (choice B) have not been implicated in AMN to our knowledge. Neuroimaging (choice D) is not indicated in AMN.

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Article Information

Corresponding Author: Rishi P. Singh, MD, Cole Eye Institute, Cleveland Clinic, 9500 Euclid Ave, I-32, Cleveland, OH 44195 (singhr@ccf.org).

Published Online: June 4, 2020. doi:10.1001/jamaophthalmol.2020.0753

Conflict of Interest Disclosures: Dr Srivastava reported receiving grants and personal fees from Novartis, Regeneron, EyePoint, Gilead, Allergan, Clearside, and Bausch & Lomb; grants from Santen; and personal fees from Zeiss, Optos, and AbbVie outside the submitted work. Dr Singh reported receiving personal fees from Regeneron, Genentech, Alcon, Novartis, and Bausch & Lomb and grants from Apellis and Graybug outside the submitted work. No other disclosures were reported.

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

References
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Sarraf  D , Rahimy  E , Fawzi  AA ,  et al.  Paracentral acute middle maculopathy: a new variant of acute macular neuroretinopathy associated with retinal capillary ischemia.   JAMA Ophthalmol. 2013;131(10):1275-1287. doi:10.1001/jamaophthalmol.2013.4056 PubMedGoogle ScholarCrossref
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Thanos  A , Faia  LJ , Yonekawa  Y , Randhawa  S .  Optical coherence tomographic angiography in acute macular neuroretinopathy.   JAMA Ophthalmol. 2016;134(11):1310-1314. doi:10.1001/jamaophthalmol.2016.3513 PubMedGoogle ScholarCrossref
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Fawzi  AA , Pappuru  RR , Sarraf  D ,  et al.  Acute macular neuroretinopathy.   Retina. 2012;32(8):1500-1513. doi:10.1097/IAE.0b013e318263d0c3 PubMedGoogle ScholarCrossref
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Rahimy  E , Kuehlewein  L , Sadda  SR , Sarraf  D .  Paracentral acute middle maculopathy.   Retina. 2015;35(10):1921-1930. doi:10.1097/IAE.0000000000000785 PubMedGoogle ScholarCrossref
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Hashimoto  Y , Saito  W , Mori  S , Saito  M , Ishida  S .  Increased macular choroidal blood flow velocity during systemic corticosteroid therapy in a patient with acute macular neuroretinopathy.   Clin Ophthalmol. 2012;6:1645-1649. doi:10.2147/OPTH.S35854PubMedGoogle Scholar
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