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Bursztyn, Lulu L. C. D. MD, MSc*,†; Belrose, Jillian C. PhD*; Coupland, Stuart G. PhD; Fraser, J. Alexander MD*,†,§; Proulx, Alain A. MD*,†

Retinal Cases & Brief Reports: April 2015 - Volume 9 - Issue 2 - p 173–176
doi: 10.1097/ICB.0000000000000131
Case Report

Purpose: To describe the clinical findings in a patient demonstrating recovery from nonparaneoplastic autoimmune retinopathy after a minimal course of steroid treatment.

Methods: Clinical presentation was documented, and paraclinical tests were obtained using Humphrey automated perimetry for visual fields, Western blotting for antiretinal antibodies, and electroretinography for evaluation of rod and cone function.

Results: Initial presentation revealed marked visual field deficits, electroretinographic dysfunction, and the presence of α-enolase autoantibodies. After a brief course of oral corticosteroids, the patient demonstrated improvement in visual fields, disappearance of α-enolase autoantibodies, partial recovery of the cone on-response, and complete recovery of the rod response.

Conclusion: This case is distinguished from previous reports by the rapidity and degree of recovery, the brevity of treatment required, and the unique electroretinographic recovery pattern with concomitant disappearance of α-enolase autoantibodies. These findings suggest a pathologic role for α-enolase autoantibodies in autoimmune rod bipolar cell dysfunction. Identification of other cases exhibiting such improvements and associated autoantibody activity may expand our understanding of nonparaneoplastic autoimmune retinopathy disease pathogenesis.

This is the first report of autoimmune retinopathy demonstrating remarkable recovery of visual fields, rod, and on-code function measured by electroretinography and an associated loss of α-enolase autoantibodies after a brief course of steroid treatment.

*Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada;

Department of Ophthalmology, Ivey Eye Institute, Western University, London, Ontario, Canada;

Ottawa Hospital Research Institute, University of Ottawa, Ottawa, Ontario, Canada; and

§Department of Clinical Neurological Sciences, Western University, London, Ontario, Canada.

Reprint requests: Lulu L. C. D. Bursztyn, MD, MSc, Ivey Eye Institute, St. Joseph's Hospital, 268 Grosvenor Street, London, Ontario, Canada N6A 4V2; e-mail:

L. L. C. D. Bursztyn and J. C. Belrose contributed equally to this work.

None of the authors have any financial/conflicting interests to disclose.

© 2015 by Ophthalmic Communications Society, Inc.