DUCHENNE MUSCULAR-DYSTROPHY - NEGATIVE SCOTOPIC BRIGHT-FLASH ELECTRORETINOGRAM BUT NOT CONGENITAL STATIONARY NIGHT BLINDNESS

Patients with Duchenne muscular dystrophy (DMD) hare recently been rep orted to have an abnormal scotopic electroretinogram (ERG) showing wea k rod-related responses along with a negative configuration of the bri ght-flash response, which has been described as being similar to the o ne in congenital stationary night blindness (CSNB), We compared qualit atively and quantitatively the ERGs of 6 subjects with DMD, 10 subject s with the complete form of CSNB (cCSNB), 13 subjects with the incompl ete form of CSNB (iCSNB) and 1 subject with complex glycerol kinase de ficiency (CGKD), The rod-related activity and the bright-flash respons es were abnormal and similar in all four groups. The cone-related acti vity, however, was within normal limits only in the DMD group; the b-w ave was subnormal in CGKD, truncated in cCSNB and nearly absent in iCS NB. The electrophysiologic signature in DMD clearly distinguishes the retinal function of these patients from any other retinal condition so far described.