Table.
Summary Data
| VMD With Normal MRI | VMD With Abnormal MRI | VMD with Metabolic Disorder | Severe CVI | All Controls | |
|---|---|---|---|---|---|
| N | 10 | 14 | 6 | 10 | 26 |
| Mean age | 2.2 (1.6) | 1.5 (1.1) | 2.5 (3.7) | 1.1 (0.6) | 2.1 (1.5) |
| logMAR | 1.9 | 1.9 | 1.4 | 2.6 | |
| Range | 3.1 to < 0.23 cy/deg | 6.5 to < 0.23 cy/deg | 9.8 to < 0.23 cy/deg | 1.3 to < 0.23 cy/deg | |
| Stable gaze | 100% | 86% | 50% | 50% | |
| Strabismus | 40% | 57% | 67% | 50% | |
| Microcephaly | 20% | 36% | 50% | 50% | |
| DD | 90% | 93% | 100% | 100% | |
| Hypotonia | 50% | 93% | 100% | 70% | |
| Seizures | 50% | 64% | 33% | 80% | |
| Check VEP† | Mean (SD) | Mean (SD) | Mean (SD) | Mean (SD) | Mean (SD) |
| Amplitude | 38.0 (20.4) | 38.2 (14.9) | 22.5 (7.7) | 5.0 (2.9)*** | 36.6 (11.2) |
| SNR | 72.4 (26.1) | 73.6 (20.3) | 68.0 (6.2) | 1.2 (0.6)*** | 112.1 (61.6) |
| Latency | 98.0 (8.3) | 114.4 (27.4) | 109.3 (16.6) | 126.4 (31.2) | 102.9 (7.2) |
| Onset VEP† | |||||
| Amplitude | 43.9 (26.4) | 34.8 (24.8) | 29.3 (11.3) | 10.9 (13.4)** | 33.8 (11.0) |
| SNR | 67.8 (19.0) | 54.6 (23.6) | 63.3 (21.0) | 12.3 (24.0)*** | 70.1 (30.6) |
| Latency | 115.0 (30.9) | 130.5 (61.7) | 103.8 (11.8) | 130.3 (35.3) | 100.4 (11.6) |
Age in years; logMAR visual acuity corrected for age; cy/deg is grating acuity in cycles/degree; microcephaly is < 5% of normative data for occipital frontal circumference.
VMD = visuomotor dysfunction; CVI = cortical visual impairment; DD = developmental delay.
†
Averaging using the FT-selective method; P values statistically significant from controls on t-test after Bonferroni correction.
** = P < 0.001.
*** = P < 0.0001.