Progression of Visual Field Defects in Leber Hereditary Optic Neuropathy: Experience of the LHON Treatment Trial
Accepted 22 December 2005. published online 06 February 2006.
Purpose
To describe the visual fields of patients with Leber hereditary optic neuropathy (LHON), a maternally 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 mutation 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 perimetry 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 deviation, 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 findings that typically worsen rapidly over weeks to months to similar central scotomatous damage. Quantitative automated static perimetry is helpful in elucidating the natural history of LHON and in understanding the underlying pathology and pathophysiology of this disease.
aDepartment of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia
bDepartment of Neurology, Emory University School of Medicine, Atlanta, Georgia
cDepartment of Neurological Surgery, Emory University School of Medicine, Atlanta, Georgia
dDepartment of Ophthalmology, University of Virginia, Charlottesville, Virginia
eDepartments of Ophthalmology, Neurology, and Neurosurgery, University of Florida, Gainesville, Florida
fSwedish Neuroscience Institute, Seattle, Washington
gDean A. McGee Eye Institute, Oklahoma City, Oklahoma
hDepartments of Ophthalmology and Visual Science and Neurology, Yale University School of Medicine, New Haven, Connecticut
iInstitute of Ophthalmology and Visual Sciences, University of Medicine and Dentistry of New Jersey, Newark, New Jersey
Inquiries to Nancy J. Newman, MD, Neuro-Ophthalmology Unit, Emory Eye Center, 1365-B Clifton Road NE, Atlanta, GA 30322
N.J.N. acted as a consultant for Allergan Inc.
Supported in part by Allergan Inc, Irvine, California. N.J.N. and V.B. were supported in part by a departmental grant (Department of Ophthalmology) 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.
ABSTRACT