Table 1.
Clinical, sleep, and brain volume data in Wolfram syndrome patients
| Mean | SD | Minimum | Maximum | N | |
|---|---|---|---|---|---|
| Age (years) | 18.0 | 5.8 | 8.9 | 29.7 | 21 |
| Diabetes Duration (years) | 12.4 | 6.3 | 0.0 | 26.5 | 21 |
| BMI | 23.2 | 5.0 | 16.1 | 33.0 | 20 |
| HbA1c (%) | 7.9 | 1.9 | 5.0 | 12.4 | 19 |
| Apnea Hypopnea Index (AHI) | 5.6 | 5.3 | 0.0 | 22.0 | 21 |
| Sleep Efficiency (%)a | 84.0 | 11.6 | 36.9 | 93.2 | 21 |
| Sleep Onset Latency (minutes)a | 35.7 | 72.6 | 3.0 | 346.5 | 21 |
| Total sleep time (minutes)a | 441.7 | 90.5 | 220.0 | 624.5 | 21 |
| PSQI Total Score | 5.7 | 3.3 | 1.0 | 12.0 | 18 |
| PSQ Total Score | 0.19 | 0.16 | 0 | 0.41 | 15 |
| ESS Total Score | 6.4 | 3.7 | 2.0 | 15.0 | 17 |
| WURS Physical Score | 11.1 | 9.7 | 1.0 | 35.0 | 21 |
| PANESS Total Score | 37.3 | 15.8 | 11.0 | 71.0 | 18 |
| Visual Acuity (Logmar) | 0.7 | 0.5 | 0.0 | 1.9 | 20 |
| Ventral Pons Volume (mm3) | 5933.9 | 1311.7 | 4316.6 | 7830.3 | 15 |
| Dorsal Pons Volume (mm3) | 2941.5 | 292.9 | 2563.6 | 3494.3 | 15 |
| Total Pons Volume (mm3) | 8875.4 | 1457.9 | 6997.6 | 10918.4 | 15 |
| Medulla Volume (mm3) | 2045.2 | 190.4 | 1751.0 | 2449.3 | 15 |
| Cerebellar White Matter Volume (mm3) | 11363.2 | 1651.9 | 8000.2 | 14355.3 | 15 |
| Cerebellar Gray Matter Volume (mm3) | 45064.8 | 4188.2 | 37680.2 | 55467.4 | 15 |
SD standard deviation, BMI body mass index, HbA1c Hemoglobin A1c, PSQI Pittsburgh Sleep Quality Index, PSQ Pediatric Sleep Questionnaire, ESS Epworth Sleepiness Scale, WURS Wolfram Unified Rating Scale, PANESS Physical and Neurological Examination for Subtle Signs. All data in Table 1 represents only Wolfram patients, and does not include controls
adata collected from Actiwatch