GYRATE ATROPHY: A RARE CASE REPORT

Abstract

Background- Gyrate atrophy of the retina & choroid is a rare autosomal recessive inherited disease, characterized by progressive chorioretinal atrophy that results in progressive deterioration of peripheral & night vision leading to blindness. Case presentation- This report presents a 40 year old woman consulting for a progressive fall of visual acuity RE with defective night vision. Ocular fundoscopy showed regions of conuent rounded chorioretinal atrophy. Visual eld & retinal angiography were altered. High levels of plasma ornithine 685 nmol/mL) was detected and a diagnosis of gyrate atrophy of retina & choroid was made. The patient was treated with high dose pyridoxine supplement (300 mg/d for 6 months) & ornithine level of her serum was successfully reduced. Conclusion- The exact mechanism of chorioretinal atrophy in hyperornithemia is not known and a small percentage of affected people respond to Vitamin B supplimentation