Case Report

Pattern Dystrophy In Curschmann Steinert Syndrome - Case Report

  • Umut Asli Dinç
  • Sinan Tatlipinar
  • Sule Ziylan
  • Levent Alimgil
  • Demir Basar

Received Date: 29.07.2008 Accepted Date: 12.09.2008 Turk J Ophthalmol 2008;38(6):524-527

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 examination 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 accumulation 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.

Keywords: Curschmann Steinert syndrome, pattern dystrophy, fundus autofluorescence

Full Text (Turkish)