Table 1.
Age group | Genotype | Visual acuity BCVAa (LogMAR) | Cone sensitivity (threshold) | fMRI | pRF sized | Post-treatment teste | |||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Left eye | Right eye | Both | Square | Ridge | Intercept | Slope | AICWb | CCFLc | |||||
Pre-treatment treated patients with ACHM | |||||||||||||
Tr1 | Child | CNGA3 | 0.94f | 0.92 | 0.86 | 0.24 | 0.30 | 125.25 | −0.14 | 0 | −0.05 | 1.69 | 6 |
Tr2 | Child | CNGA3 | 0.76 | 0.80f | 0.70 | 0.46 | N/A | 32.29 | 0.02 | 0 | −0.03 | 1.91 | 8 |
Tr3 | Child | CNGB3 | 0.80 | 0.84f | 0.84 | 0.34 | 0.34 | 49.36 | 0.11 | 0 | −0.03 | 1.68 | 14 |
Tr4 | Child | CNGA3 | 0.78f | 0.76 | 0.78 | 0.34 | 0.38 | 33.15 | 0.17 | 0 | 0.12 | 1.45 | 14 |
Post-treatment treated patients with ACHM | |||||||||||||
Tr1 | Child | CNGA3 | 0.92f | 0.84 | 0.82 | 0.16 | 0.16 | −16.74 | 0.97 | 1 | 0.32 | 1.4 | |
Tr2 | Child | CNGA3 | 0.76 | 0.86f | 0.72 | 0.16 | 0.16 | −8.27 | 0.82 | 1 | 0.60 | 1.76 | |
Tr3 | Child | CNGB3 | 0.56 | 0.72f | 0.54 | 0.34 | 0.34 | 32.04 | −0.12 | 0 | −0.11 | 1.47 | |
Tr4 | Child | CNGA3 | 0.78f | 0.72 | 0.70 | 0.34 | 0.34 | −2.00 | −0.06 | 0 | 0.02 | 1.31 | |
Untreated patients with ACHM | |||||||||||||
Utr1 | Child | CNGB3 | 1.04 | 1.00 | 0.94 | 0.34 | 0.46 | 43.78 | 0.29 | 0 | −0.31 | 2.27 | |
Utr2 | Child | CNGB3 | 0.96 | 0.94 | 0.82 | N/A | N/A | 129.82 | −0.08 | 0 | −0.75 | 1.79 | |
Utr3 | Child | CNGB3 | 0.84 | 0.94 | 0.82 | 0.42 | 0.38 | −422.50 | −17.36 | 0 | −0.03 | 1.86 | |
Utr4 | Child | CNGB3 | 0.90 | 0.90 | 0.86 | 0.34 | 0.30 | 85.97 | 0.29 | 0 | 0.70 | 1.46 | |
Utr5 | Child | CNGB3 | 0.86 | 0.96 | 0.90 | 0.52 | 0.38 | 32.74 | 0.50 | 0 | 0.12 | 1.53 | |
Utr6 | Child | CNGA3 | 0.86 | 0.92 | 0.92 | 0.46 | N/A | −35.42 | −4.64 | 0 | 0.12 | 2.3 | |
Utr7 | Child | CNGA3 | 0.76 | 0.72 | 0.72 | 0.38 | 0.30 | 64.89 | 2.02 | 0 | 0.11 | 1.2 | |
Utr8 | Adult | CNGA3 | 0.70 | 0.70 | 0.70 | 0.52 | 0.34 | −3 × 106 | −2 × 104 | 0 | 0.41 | 1.66 | |
Utr9 | Adult | CNGA3 | 0.90 | 0.80 | 0.84 | 0.46 | 0.38 | −97.29 | 0.50 | 0 | −0.03 | 1.42 | |
Normal-sighted controls | |||||||||||||
C1 | Child | Normalg | N/A | N/A | 0.02 | 0.16 | 0.16 | 2.20 | 1 | 1 | 0.86 | 1.92 | |
C2 | Child | Normalg | N/A | N/A | −0.04 | 0.16 | 0.16 | −4.97 | 1 | 1 | 0.74 | 1.56 |
N/A indicates missing data. Measures from Patients Tr1 and Tr2 are presented using bold text. AICW = Akaike iInformation Criterion Weight; CCFL = Fisher–Lee correlation coefficient.
Best corrected visual acuity.
Akaike weight.
Fisher–Lee correlation coefficient.
Rod mean pRF size.
Duration between the pre- and post-treatment behavioural and fMRI measures (in months).
Treated eye.
Normal-sighted control with no ACHM.