Table 3.
Baseline clinical information per subject category (core assessments)
| Measure | HDGECs (N = 1,071) | Control (N = 463) | Manifest (N = 739) | Premanifest (N = 332) | Genotype Negative (N = 197) | Family Control (N = 266) |
|---|---|---|---|---|---|---|
| Core clinical assessments | ||||||
| UHDRS Motor scale | ||||||
| n | 1,069 | 463 | 737 | 332 | 197 | 266 |
| Mean ± SD | 26.9 ± 22.3 | 1.9 ± 3.5 | 37.4 ± 19.0 | 3.5 ± 4.4 | 2.3 ± 4.2 | 1.6 ± 2.9 |
| Median (range) | 25.0 (0.0; 97.0) | 0.0 (0.0; 28.0) | 35.0 (3.0; 97.0) | 2.0 (0.0; 32.0) | 1.0 (0.0; 28.0) | 0.0 (0.0; 17.0) |
| UHDRS TFC | ||||||
| n | 1,071 | 463 | 739 | 332 | 197 | 266 |
| Mean ± SD | 9.5 ± 3.6 | 12.8 ± 0.7 | 8.1 ± 3.5 | 12.8 ± 0.8 | 12.8 ± 1.0 | 12.9 ± 0.4 |
| Median (range) | 11.0 (0.0; 13.0) | 13.0 (5.0; 13.0) | 8.0 (0.0; 13.0) | 13.0 (5.0; 13.0) | 13.0 (5.0; 13.0) | 13.0 (9.0; 13.0) |
| UHDRS FAS | ||||||
| n | 1,051 | 463 | 723 | 328 | 197 | 266 |
| Mean ± SD | 20.2 ± 6.2 | 24.9 ± 0.8 | 18.2 ± 6.4 | 24.8 ± 0.8 | 24.8 ± 1.1 | 24.9 ± 0.5 |
| Median (range) | 23.0 (0.0; 25.0) | 25.0 (14.0; 25.0) | 20.0 (0.0; 25.0) | 25.0 (17.0; 25.0) | 25.0 (14.0; 25.0) | 25.0 (20.0; 25.0) |
| UHDRS Independence | ||||||
| n | 1,071 | 463 | 739 | 322 | 197 | 266 |
| Mean ± SD | 84.3 ± 17.7 | 99.6 ± 2.8 | 77.5 ± 17.4 | 99.4 ± 2.9 | 99.3 ± 4.1 | 99.8 ± 1.2 |
| Median (range) | 90.0 (15.0; 100) | 100.0 (55.0; 100) | 80.0 (15.0; 100.0) | 100.0 (70.0; 100) | 100.0 (55.0; 100) | 100.0 (90.0; 100) |
| PBA‐s depression score | ||||||
| n | 1,061 | 463 | 730 | 331 | 197 | 266 |
| Mean ± SD | 4.3 ± 6.1 | 2.7 ± 4.7 | 4.5 ± 5.9 | 3.9 ± 6.4 | 2.7 ± 5.2 | 2.7 ± 4.2 |
| Median (range) | 2.0 (0.0; 48.0) | 0.0 (0.0; 30.0) | 2.0 (0.0; 38.0) | 1.0 (0.0; 48.0) | 0.0 (0.0; 30.0) | 1.0 (0.0; 24.0) |
| PBA‐s irritability score | ||||||
| n | 1,067 | 463 | 735 | 332 | 197 | 266 |
| Mean ± SD | 2.5 ± 4.2 | 1.1 ± 2.6 | 2.7 ± 4.4 | 1.9 ± 3.7 | 1.2 ± 3.0 | 1.1 ± 2.2 |
| Median (range) | 0.0 (0.0; 28.0) | 0.0 (0.0; 21.0) | 1.0 (0.0; 25.0) | 0.0 (0.0; 28.0) | 0.0 (0.0; 21.0) | 0.0 (0.0; 15.0) |
| PBA‐s psychosis score | ||||||
| n | 1,064 | 463 | 733 | 331 | 197 | 266 |
| Mean ± SD | 0.2 ± 1.2 | 0.1 ± 1.2 | 0.2 ± 1.4 | 0.0 ± 0.4 | 0.2 ± 1.8 | 0.0 ± 0.6 |
| Median (range) | 0.0 (0.0; 18.0) | 0.0 (0.0; 18.0) | 0.0 (0.0; 18.0) | 0.0 (0.0; 4.0) | 0.0 (0.0; 18.0) | 0.0 (0.0; 9.0) |
| PBA‐s apathy score | ||||||
| n | 1,064 | 463 | 732 | 332 | 197 | 266 |
| Mean ± SD | 2.7 ± 4.2 | 0.4 ± 1.6 | 3.4 ± 4.6 | 1.0 ± 2.4 | 0.5 ± 1.7 | 0.4 ± 1.6 |
| Median (range) | 0.0 (0.0; 16.0) | 0.0 (0.0; 12.0) | 1.0 (0.0; 16.0) | 0.0 (0.0; 16.0) | 0.0 (0.0; 12.0) | 0.0 (0.0; 12.0) |
| PBA‐s executive function score | ||||||
| n | 1,064 | 463 | 732 | 332 | 197 | 266 |
| Mean ± SD | 2.4 ± 4.4 | 0.5 ± 2.4 | 3.0 ± 4.8 | 1.1 ± 3.0 | 0.6 ± 2.6 | 0.4 ± 2.3 |
| Median (range) | 0.0 (0.0; 32.0) | 0.0 (0.0; 25.0) | 0.0 (0.0; 32.0) | 0.0 (0.0; 32.0) | 0.0 (0.0; 25.0) | 0.0 (0.0; 24.0) |
| Symbol Digit Modalities Test (total correct) | ||||||
| n | 1,027 | 459 | 696 | 331 | 195 | 264 |
| Mean ± SD | 32.4 ± 17.5 | 50.7 ± 12.4 | 24.0 ± 12.7 | 50.0 ± 12.1 | 53.2 ± 12.6 | 48.9 ± 12.0 |
| Median (range) | 30.0 (0.0; 90.0) | 51.0 (0.0; 91.0) | 23.0 (0.0; 90.0) | 51.0 (10.0; 84.0) | 54.0 (0.0; 91.0) | 50.0 (1.0; 88.0) |
| Stroop Color Naming (total correct) | ||||||
| n | 1,049 | 457 | 717 | 332 | 193 | 264 |
| Mean ± SD | 52.8 ± 22.6 | 75.6 ± 15.7 | 42.8 ± 18.2 | 74.3 ± 14.6 | 77.5 ± 18.0 | 74.2 ± 13.6 |
| Median (range) | 52.0 (0.0; 130.0) | 76.0 (0.0; 132.0) | 43.0 (0.0; 130.0) | 74.5 (34.0; 109.0) | 80.0 (0.0; 132.0) | 75.0 (45.0; 129.0) |
| Stroop Word Reading (total correct) | ||||||
| n | 1,052 | 457 | 720 | 332 | 192 | 265 |
| Mean ± SD | 67.6 ± 28.0 | 94.1 ± 18.0 | 56.2 ± 23.9 | 92.4 ± 18.7 | 95.8 ± 20.6 | 92.9 ± 15.9 |
| Median (range) | 67.0 (0.0; 199.0) | 96.0 (0.0; 148.0) | 56.0 (0.0; 199.0) | 95.0 (6.0; 149.0) | 100.0 (0.0; 148.0) | 93.0 (37.0; 145.0) |
| Categorical Verbal Fluency test (total correct) | ||||||
| n | 1,049 | 460 | 720 | 329 | 195 | 265 |
| Mean ± SD | 14.8 ± 7.2 | 20.8 ± 5.7 | 11.9 ± 5.7 | 21.1 ± 6.2 | 21.0 ± 6.1 | 20.7 ± 5.3 |
| Median (range) | 14.0 (0.0; 40.0) | 21.0 (3.0; 41.0) | 11.0 (0.0; 32.0) | 20.0 (3.0; 40.0) | 21.0 (3.0; 41.0) | 21.0 (8.0; 40.0) |
UHDRS '99 = Unified Huntington's Disease Rating Scale (1999 version).