TABLE 2.
Clinical severity measures in individuals with Wolfram syndrome.
| TP 1 | N with 1 TP | TP 1a | TP 2 | N with 2 TPs | |
| Disease duration (years) | 4.5 ± 4.4 | 37 | 3.9 ± 4.1 | 5.6 ± 4.0 | 26 |
| Plasma glucose (mg/dL) | 187.8 ± 68.4 | 36 | 187.7 ± 72.5 | 181.6 ± 84.4 | 26 |
| HbA1c | 7.5 ± 1.5 | 36 | 7.5 ± 1.7 | 7.3 ± 1.1 | 26 |
| WURS Physical Activity subscale score | 5.0 ± 5.7 | 34 | 4.3 ± 3.2 | 4.5 ± 3.7 | 24 |
| UPSIT total score (number correct) | 24.9 ± 7.5 | 37 | 24.9 ± 7.8 | 24.0 ± 7.3 | 27 |
| Visual acuity (logMAR) | 0.56 ± 0.45 | 34 | 0.50 ± 0.34 | 0.56 ± 0.39* | 27 |
| Color vision (number correct) | 9.2 ± 9.3 | 30 | 10.7 ± 9.4 | 10.2 ± 10 | 20 |
| Ventral pons volume (mm3) | 6280 ± 1160 | 27 | 6133 ± 1190 | 6030 ± 1249* | 20 |
| Brainstem volume (mm3) | 15079 ± 1590 | 18 | 15079 ± 1590 | 14879 ± 1649** | 18 |
| Cerebellar cortex volume (mm3) | 46109 ± 4440 | 26 | 45573 ± 4393 | 44712 ± 4420** | 19 |
| Thalamus volume (mm3) | 6530 ± 478 | 26 | 6546 ± 381 | 6429 ± 425** | 19 |
| Follow-up duration (years, range) | NA | NA | NA | 1.8 (0.99–2.0) | 27 |
Mean (SD) shown except where noted.
TP, time point; NA, not applicable; WURS, Wolfram United Rating Scale; UPSIT, University of Pennsylvania Smell Identification Test.
**, *p < 0.01, 0.05 relative to time point 1.
aColumn includes individuals with Wolfram syndrome who had data from both time points.