Experience of the LHON Treatment Trial

Progression of Visual Field Defects in Leber Hereditary Optic Neuropathy: Experience of the LHON Treatment Trial

Nancy J. Newman MD^{a, b, c, ,} , Valérie Biousse MD^a, b, Steven A. Newman MD^d, M. Tariq Bhatti MD^e, Steven R. Hamilton MD^f, Bradley K. Farris MD^g, Robert L. Lesser MD^h and Roger E. Turbin MDⁱ

^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, ConnecticutⁱInstitute of Ophthalmology and Visual Sciences, University of Medicine and Dentistry of New Jersey, Newark, New Jersey

Accepted 22 December 2005.  

Available online 8 June 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.