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. 2010 Jun 2;18(10):1121–1126. doi: 10.1038/ejhg.2010.81

Figure 2.

Figure 2

(a) Colour fundus pictures of patient 509 showing the posterior pole (left eye) with macular atrophy and chorioretinal atrophy starting from midperipheral region of the retina (i), and the nasal region (left eye) with peripheral chorioretinal atrophy (seven-dot star), sparing midperipheral retina (four-dot star). The arrows disclose the sharp margin of normal-appearing and atrophic retina (ii). (b) Colour fundus pictures of patient 512 showing the posterior pole (right eye) with macular atrophy, retinal vessel attenuation and severe peripheral chorioretinal atrophy with bone spicule pigmentation, sparing midperipheral retina (iii), and the posterior pole (left eye) with macular atrophy, retinal vessel attenuation and severe peripheral chorioretinal atrophy with bone spicule pigmentation, sparing midperipheral retina (iv). (c) Cone ERG of patient 512 disclosing subnormal response (upper recording right eye and lower recording left eye) (v) and rod ERG of the same patient disclosing subnormal response (upper recording right eye, lower recording left eye; arrow a represents a-wave, and arrow b represents b-wave of rod ERG; vi).