Table 3. Baseline motor, behavioral, and cognitive characteristics of the COHORT population by group.
| Groups that carry an expanded allele | Groups that do not carry an expanded allele | ||||||
| Individuals with clinically diagnosed HD[n = 930] | First-degree relatives who pursued genetic testing and carry an expanded allele[n = 248] | First-degree relatives who did not pursue genetic testing and carry an expanded allele[n = 112] | First-degreerelatives who did not pursue genetic testing and do not carry an expanded allele[n = 224] | First-degree relatives who pursued genetic testing and do not carry an expanded allele[n = 41] | Spousesand caregivers[n = 430] | P-value* | |
| Motor assessment ** | |||||||
| Total UHDRS motor assessment [0–124] | 39.1 (18.3) | 6.8 (10.6) | 6.9 (11.3) | 2.3 (3.3) | 1.2 (2.5) | 1.5 (2.8) | <0.001 |
| Total maximal chorea score [0–28] | 10.1 (5.0) | 1.4 (3.2) | 2.0 (4.2) | 0.2 (0.6) | 0.07 (0.4) | 0.03 (0.3) | <0.001 |
| Total maximal dystonia score [0–20] | 3.5 (3.8) | 0.4 (1.5) | 0.3 (1.2) | 0.03 (0.2) | 0 (0) | 0.02 (0.1) | <0.001 |
| Behavioral assessment | |||||||
| UHDRS Behavioral frequency [0–44] | 7.0 (6.1) | 6.9 (6.9) | 6.4 (6.2) | 4.5 (4.5) | 4.9 (4.7) | 4.4 (4.2) | <0.001 |
| UHDRS Behavioral frequency×severity [0–176] | 14.0 (15.4) | 12.7 (17.7) | 11.2 (15.2) | 6.5 (9.1) | 7.8 (12.5) | 6.2 (9.0) | <0.001 |
| Cognitive assessment | |||||||
| Mini Mental State Examination [0–30] | 25.0 (4.6) | 28.6 (1.9) | 28.2 (2.5) | 29.0 (1.6) | 29.4 (1.3) | 29.1 (1.5) | <0.001 |
| UHDRS Verbal fluency | 21.9 (12.5) | 38.1 (14.1) | 36.6 (14.9) | 39.8 (11.7) | 42.2 (10.2) | 40.1 (12.1) | <0.001 |
| UHDRS Symbol digit modalities test | 23.6 (11.2) | 45.4 (12.5) | 45.2 (12.4) | 49.5 (11.8) | 49.2 (10.4) | 45.9 (10.2) | <0.001 |
| UHDRS Stroop color naming | 44.3 (16.9) | 70.9 (17.8) | 71.1 (16.0) | 76.9 (15.8) | 72.8 (14.5) | 73.3 (14.8) | <0.001 |
| UHDRS Stroop word reading | 58.0 (21.9) | 90.1 (21.5) | 90.4 (19.2) | 95.8 (16.3) | 99.2 (17.8) | 94.6 (19.3) | <0.001 |
| UHDRS Stroop interference | 25.5 (12.5) | 42.8 (12.9) | 41.2 (12.3) | 44.8 (12.0) | 50.6 (23.7) | 41.8 (12.1) | <0.001 |
| UHDRS Independence assessment | 79.1 (16.2) | 97.2 (7.2) | 99.1 (3.9) | 99.9 (0.9) | 99.5 (3.1) | 99.8 (1.4) | <0.001 |
| UHDRS Functional assessment | 18.7 (6.0) | 24.4 (1.9) | 24.8 (1.1) | 24.9 (0.3) | 24.9 (0.5) | 24.9 (1.3) | <0.001 |
| UHDRS Total functional capacity | 8.1 (3.4) | 12.3 (1.7) | 12.7 (1.0) | 12.9 (1.0) | 12.9 (0.5) | 12.9 (0.6) | <0.001 |
Values are listed as mean (standard deviation) unless otherwise noted.
COHORT = Cooperative Huntington Observational Research Trial; HD = Huntington disease; (CAG)n = cytosine-adenine-guanine repeat length in the Huntingtin allele; UHDRS = Unified Huntington's Disease Rating Scale.
*P-values refer to overall tests of heterogeneity across all six groups.
**For motor and behavioral measures, higher scores reflect greater impairment. For cognitive, independence, and functional measures, higher scores reflect less impairment.