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Muriel I. Kaiser-Kupfer, MD; Rafael C. Caruso, MD; David Valle, MD; George F. Reed, PhD

Arch Ophthalmol. 2004;122:982-984.

Objective  To quantify the effect of long-term reduction of plasma ornithine levels through adherence to an arginine-restricted diet on visual function in patients of all ages with gyrate atrophy of the retina and choroid.

Methods  A long-term observational study was conducted on 27 patients with gyrate atrophy, 17 of whom elected to comply with the arginine-restricted diet and 10 who were unable to comply. The mean rates of change in the electroretinogram combined response, electroretinogram flicker response, and kinetic and static perimetry were determined.

Results  After mean follow-up of 13.9 years for the patients on the diet and 14.1 years for those not on the diet, the mean rates of change for the diet group compared with those of the no-diet group were statistically significantly slower for all outcome measures (age-adjusted P<.05) except for static perimetry (P = .06).

Conclusions  Adhering to an arginine-restricted diet so as to lower the plasma ornithine level below an average of 5.29 to 6.61 mg/dL (400-500 µmol/L) will slow the loss of function as measured by sequential electroretinography and visual field examinations.

From the Ophthalmic Genetics and Visual Function Branch (Drs Kaiser-Kupfer and Caruso) and Biometry Branch, Division of Epidemiology and Clinical Research (Dr Reed), National Eye Institute, National Institutes of Health, Bethesda, Md; and Howard Hughes Medical Institute, The Johns Hopkins School of Medicine, Baltimore, Md (Dr Valle). Dr Kaiser-Kupfer is now retired. The authors have no relevant financial interest in this article.

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