TABLE 1:
Demographic parameters and clinical assessment scores of controls and CAG-expanded HTT allele carriers (HD group
Controls | All HD | > 8yr pre-HD | < 8yr pre-HD | Early HD | |
---|---|---|---|---|---|
Number | 16 | 24 | 9 | 6 | 9 |
Sex (male/female) | 8:8 | 13:11 | 6:3 | 1:5 | 6:3 |
Age (yr) | 44.8 ± 15.8 | 42.5 ± 12.9 | 34.4 ± 10.9 | 41.7 ± 11.5 | 50.8 ± 10.9 |
Education (yr) | 16.1 ± 2.0 | 15.3 ± 2.2 | 14.7 ± 1.9 | 16.8 ± 2.6 | 14.9 ± 2.0 |
CAG length | -- | 43.8 ± 4.3 | 41.8 ± 2.8 | 44.0 ± 4.2 | 45.6 ± 5.1 |
CAPs score | -- | 0.9 ± 0.4 | 0.6 ± 0.2 | 1.0 ± 0.1 | 1.3 ± 0.2 |
UHDRS Motor score | -- | 15.9 ± 17.4 | 4.3 ± 4.3 | 14.2 ± 11.8 | 28.6 ± 20.8 |
UHDRS Function score | 24.9 ± 0.5 | 23.3 ± 3.5* | 25.0 ± 0.0 | 23.0 ± 4.0 | 21.7 ± 4.4* |
UHDRS Behavior score | 3.5 ± 4.7 | 9.6 ± 11.2* | 7.0 ± 5.7 | 7.7 ± 4.5 | 13.6 ± 16.9 |
Total functional capacity | 13 ± 0.0 | 11.5 ± 3.0* | 12.9 ± 0.3 | 11.0 ± 3.3 | 10.3 ± 3.9 |
Montreal Cognitive Assessment | 29.6 s 0.7 | 28.2 ± 2.8* | 29.6 ± 0.2 | 28.2 ± 2.7 | 26.8 ± 3.6* |
Symbol-Digit Modalities Test score | -- | 51.5 ± 22.1 | 58.1 ± 16.9 | 54.0 ± 19.8 | 42.1 ± 27.7 |
Verbal Fluency Test score | 47.4 s 12.8 | 41.3 ± 17.4 | 50.1 ± 11.8 | 42.5 ± 16.9 | 30.5 ± 18.7* |
NOTES: CAPs score = CAG-age product scaled score; UHDRS = Unified Huntington’s Disease Rating Scale; Data are presented as means ± standard deviation
Significant difference as compared to controls with p < 0.05.