Progression of Visual Field Defects in Leber Hereditary Optic Neuropathy: Experience of the LHON Treatment Trial NANCY J. NEWMAN, MD, VALÉRIE BIOUSSE, MD, STEVEN A. NEWMAN, MD, M. TARIQ BHATTI, MD, STEVEN R. HAMILTON, MD, BRADLEY K. FARRIS, MD, ROBERT L. LESSER, MD, AND ROGER E. TURBIN, MD ● PURPOSE: To describe the visual fields of patients with Leber hereditary optic neuropathy (LHON), a mater- nally inherited disorder characterized by bilateral, often sequential vision loss, before and during progressive visual deterioration. ● DESIGN: Prospective longitudinal follow-up of serial visual fields in patients enrolled onto an open-label, nonrandomized pilot study of topical brimonidine purite as prophylactic treatment after first eye involvement in LHON. ● METHODS: Nine molecularly confirmed primary muta- tion patients with LHON with monocular vision loss for less than six months and normal visual function in the other eye were followed prospectively for up to two years. Visual fields were performed on automated perim- etry at baseline and on many follow-up visits. ● RESULTS: Despite normal visual acuity at baseline in all patients, seven patients had some minimal changes in the central visual field of the second eye. All patients had subsequent deterioration of visual acuity, mean devia- tion, and foveal sensitivity in their second eye. The earliest pattern of abnormality was typically a cecocentral defect enlarging to become a central defect, often with a superior or inferior predilection. The visual field defects in the two eyes of any given patient were remarkably similar. ● CONCLUSIONS: LHON may be a bilateral condition at onset more frequently than appreciated. Automated static perimetry of the “normal” eye may reveal subclinical find- ings that typically worsen rapidly over weeks to months to similar central scotomatous damage. Quantitative auto- mated static perimetry is helpful in elucidating the natural history of LHON and in understanding the underlying pathology and pathophysiology of this disease. (Am J Ophthalmol 2006;141:1061–1067. © 2006 by Elsevier Inc. All rights reserved.) L EBER HEREDITARY OPTIC NEUROPATHY (LHON) IS A maternally inherited bilateral optic neuropa- thy. 1–3 Central visual function is primarily af- fected, with visual acuities typically deteriorating to worse than 20/200. With rare exceptions, all patients with visual loss in one eye will have second eye involvement within one year. The classic LHON visual field defect is the central or cecocentral scotoma, suggesting predominant involvement of the papillo- macular bundle. Most studies that elaborate on the visual field abnormalities in patients with LHON eval- uated these patients once substantial visual loss had occurred. Few, if any, articles report the earliest visual field findings in patients with LHON. The recent LHON Treatment Trial provided a unique opportunity to document the very earliest visual field abnormalities in LHON and to follow the progression and evolution of these defects in individual patients. 4 Accepted for publication Dec 22, 2005. From the Departments of Ophthalmology (N.J.N., V.B.), Neurology (N.J.N., V.B.), and Neurological Surgery (N.J.N.), Emory University School of Medicine, Atlanta, Georgia; Department of Ophthalmology, University of Virginia, Charlottesville, Virginia (S.A.N.); Departments of Ophthalmology, Neurology, and Neurosurgery, University of Florida, Gainesville, Florida (M.T.B.); Swedish Neuroscience Institute, Seattle, Washington (S.R.H.); Dean A. McGee Eye Institute, Oklahoma City, Oklahoma (B.K.F.); Departments of Ophthalmology and Visual Science and Neurology, Yale University School of Medicine, New Haven, Connecticut (R.L.L.); and the Institute of Ophthalmology and Visual Sciences, University of Medicine and Dentistry of New Jersey, Newark, New Jersey (R.E.T.). Supported in part by Allergan Inc, Irvine, California. N.J.N. and V.B. were supported in part by a departmental grant (Department of Ophthal- mology) from Research to Prevent Blindness Inc, New York, New York, and by core grant P30-EY06360 (Department of Ophthalmology) from the National Institutes of Health, Bethesda, Maryland. N.J.N. is a recipient of a Research to Prevent Blindness Lew R. Wasserman Merit Award. R.E.T.’s work was supported by the Gene C. Coppa Memorial Fund. N.J.N. acted as a consultant for Allergan Inc. Inquiries to Nancy J. Newman, MD, Neuro-Ophthalmology Unit, Emory Eye Center, 1365-B Clifton Road NE, Atlanta, GA 30322; e-mail: ophtnjn@emory.edu © 2006 BY ELSEVIER INC.ALL RIGHTS RESERVED. 0002-9394/06/$32.00 1061 doi:10.1016/j.ajo.2005.12.045