Progreion of Viual Field Defect in Leber Hereditary Optic Neuropathy: Experience of the LHON Treatment Trial

Purpoe To decribe the viual field of patient with Leber hereditary optic neuropathy (LHON), a maternally inherited diorder characterized by bilateral, often equential viion lo, before and during progreive viual deterioration. Deign Propective longitudinal follow-up of erial viual field in patient enrolled onto an open-label, nonrandomized pilot tudy of topical brimonidine purite a prophylactic treatment after firt eye involvement in LHON. Method Nine molecularly confirmed primary mutation patient with LHON with monocular viion lo for le than ix month and normal viual function in the other eye were followed propectively for up to two year. Viual field were performed on automated perimetry at baeline and on many follow-up viit. Reult Depite normal viual acuity at baeline in all patient, even patient had ome minimal change in the central viual field of the econd eye. All patient had ubequent deterioration of viual acuity, mean deviation, and foveal enitivity in their econd eye. The earliet pattern of abnormality wa typically a cecocentral defect enlarging to become a central defect, often with a uperior or inferior predilection. The viual field defect in the two eye of any given patient were remarkably imilar. Concluion LHON may be a bilateral condition at onet more frequently than appreciated. Automated tatic perimetry of the “normal” eye may reveal ubclinical finding that typically woren rapidly over week to month to imilar central cotomatou damage. Quantitative automated tatic perimetry i helpful in elucidating the natural hitory of LHON and in undertanding the underlying pathology and pathophyiology of thi dieae.