Gyrate atrophy of the choroid and retina: improved visual function following reduction of plasma ornithine by diet

Abstract

In a patient with gyrate atrophy of the choroid and retina, an arginine-deficient diet has reduced plasma ornithine concentration fivefold during the past 20 months. Subjective improvement in her visual function was noted approximately 15 months after institution of her diet. This has been documented by improvements in the electroretinogram, dark-adaptation, and color vision. The improvement involves rod and, to a lesser extent, cone function. The results, although preliminary and limited to a single patient, suggest that reduction of plasma ornithine with a low arginine diet is beneficial in this disease.