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M Ikejiri, E Sato, A Mizota, E Adachi-Usami; Progress of Gyrate Atrophy of the Choroid and Retina for 21 Years . Invest. Ophthalmol. Vis. Sci. 2002;43(13):1161.
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Purpose:Gyrate atrophy (GA) is a genetic disease associated with hyperornithinemia induced by a deficiency of ornithine aminotransferase (OAT), and is characterized by progressive choroidal and retinal dystrophy that usually begins in childhood with myopia, night blindness, and age-related constriction of the visual fields. Considerable variability is observed in the age when the visual acuity (VA) begins to decrease. We report a case of GA with supplementation with high doses of pyridoxine for the 21 years. Methods:The VA, visual fields, fundus photographs, color vision, ERGs, and VEPs of a female GA patient were followed from 14 to 34-years-of-age. Results:At 14 years, her corrected VA was 20/20 x -3.5 D OD, and 20/25 x -4.0 D OS. At 34 years, her corrected VA was 20/20 x -5.0 D OD, and 20/30 x -5.0 D OS. Mild posterior polar cataracts were noted bilaterally at age 31 years. Single flash ERGs were non-recordable but the VEPs were almost normal throughout the follow-up period. Color vision was normal. Fundus photographs revealed slight progression of the chorioretinal atrophy, but the visual fields were not changed throughout the follow-up period. No significant improvement in serum ornithine levels was found after supplementation with high doses of pyridoxine (600 mg/day) but without supplementation for one month, the serum level was elevated. Conclusion:The absence of a constriction of the visual field and the slow progressive changes in visual function for the 21 years is different from other cases and may be due to the supplementation with high doses of pyridoxine.
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