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. Author manuscript; available in PMC: 2020 Mar 30.
Published in final edited form as: Expert Opin Orphan Drugs. 2020 Mar 2;8(2-3):67–78. doi: 10.1080/21678707.2020.1735352

Figure 3.

Figure 3

ISCEV Standard ERG of a 10 year-old boy with a strong family history of PRPF31 associated RP. Normal values of a healthy control are shown on the left columns. The right columns show the ERG of the 10 year-old boy. The dark-adapted rod flash (0.01 cd*s/m2) shows barely measurable components. The mixed standard flash (3 cd*s/m2) reveals reduced amplitudes, whilst peak times are normal. The third row shows barely recordable dark-adapted (3 cd*s/m2) oscillatory potentials. The light-adapted 30 Hz flicker ERG and the cone standard flash (3 cd*s/m2) both show markedly reduced responses. In summary, the ERG confirms rod and cone dysfunction in both eyes. Overall rods seem to be more affected than cones at this stage.