Introduction 1 4 5 5 13 14 5 5 6 15 16 17 20 21 13 22 GUCA1A 23 GUCY2D 24 RIMS1 25 26 RPGR ORF15 26 27 KCNV2 28 29 The main aims of the current study were to review the electrophysiological phenotypes associated with annular AF abnormalities, and to examine two heterogeneous groups of patients in more detail by comparing indices of macular function associated with abnormal macular AF in rod-cone and cone-rod dystrophies. Materials and methods 5 30 17 21 23 25 29 RPGR ORF15 RIMS1 GUCA1A KCNV2 RS1 31 32 17 33 34 18 35 36 Results 1 2 4 8 2 7 8 4 5 Fig. 1 a c a b e  Fig. 2 RPGR a RIMS1 b c RS1 d e  1 2 1 1 1 1 2 RPGR 2 RIMS1 2 KCNV2 2 2 3 P 4 4 4 4 4 5 6 Fig. 3 Comparison of mean ring radius with PERG P50 in 62 patients with rod-cone dystrophy (RP; broken linear regression line) and normal visual acuity and in 19 patients with other retinal dystrophies, including 4 cone or cone-rod dystrophy cases in which there was a detectable PERG (solid regression line)  Fig. 4 a b c d e b d e  Fig. 5 a d b, e c, f a c d f e 18 a d  Fig. 6 AF images, mfERGs and corresponding Humphrey visual fields in 3 patients with RP and normal visual acuity  GUCA1A RPGR RIMS1 3 3 RIMS1 7 7 7 7 7 RIMS1 RPGR 8 2 8 Fig. 7 a b c d e f RIMS1  Fig. 8 RPGR RIMS1 a c d f  Discussion 4 5 25 27 As patients with RP or different forms of retinal dystrophy can have indistinguishable AF abnormalities, the AF appearance cannot be used to establish a diagnosis in such cases. Non-specific annular increases in AF may be associated with a wide variety of distinctive or pathognomonic full-field ERG changes that are essential for accurate diagnosis and functional phenotyping (see below). 17 18 20 37 18 37 GUCA1A 23 GUCY2D 24 RPGR 25 26 RIMS1 26 27 KCNV2 28 RS1 22 21 13 10 26 26 27 RPGR RIMS1 26 37 RPGR 38 39 14 40 41 42 15 16 1 2 GUCA1A; 23 28 2 KCNV2 28 29 2 22 43 Conclusions A parafoveal ring of high density autofluorescence is a non-specific manifestation seen in different retinal dystrophies. Electrophysiology remains essential for accurate diagnosis. The high correlation of autofluorescence with PERG, mfERG and FMM demonstrates that AF abnormalities have functional significance and may be an important parameter in the monitoring of these patients. Autofluorescence may be of prognostic value and may help identify suitable patients and retinal areas amenable to future therapeutic intervention.