ABSTRACT

A case of butterfly shaped pattern dystrophy of macula is described in a patient having Curschmann Steinert syndrome (Myotonic dystrophy type 1). A seventy-three years-old man having myotonic muscular dystrophy referred our clinic for visual acuity decrease in both eyes. Ophthalmological exa.minat.ion revealed a best corrected visual acuity level of 0.7 in both eyes. Retinal pigment epithelium changes at macula and cellophane maculopathy were detected in both eyes during fundus examination. Hyperautofluorescence secondary to lipofuscin accumu-lation in retinal pigment epithelium cells and hypoautofluorescence related to the atrophy of retinal pigment epithelium cells were found in fundus autofluorescence imaging. Pattern dystrophy related to Curschmann Steinert syndrome may be easily detected by fundus autofluorescence imaging without fundus fluorescein angiography and other ancillary tests.