From: Endophenotypical drift in Huntington’s disease: a 5-year follow-up study
Premanifest HD gene-expansion carriers | Motor manifest HD gene-expansion carriers | Level of significance p values | Premanifest HD gene-expansion carriers | Motor manifest HD gene-expansion carriers | Level of significance p values | |
---|---|---|---|---|---|---|
Cohort 2013 | Cohort 2018 | |||||
Number | 51 | 56 | 30 | 44 | ||
Gender m/f | 30/21 | 33/23 | 0.32 | 17/13 | 25/19 | 1.0 |
Age at examination (years) | 36 (20–54) | 50 (24–75) | < 0.001 | 40.5 (30–60) | 53.5 (29–80) | 0.0002 |
CAG repeat length | 42 (39–48) | 43 (40–53) | < 0.38 | 41 (39–48) | 43 (40–53) | 0.0052 |
Disease Burden scores | 234.0 (108.0–437.0) | 241.3 (133.0–437.5) | ||||
UHDRS-motor (score) | 2 (0–5) | 21 (6–51) | < 0.001 | 1 (0–5) | 32 (7–91) | < 0.0001 |
TFC (score) | 13 (11–13) | 10 (4–13) | < 0.001 | 13 (11–13) | 8 (0–13) | < 0.0001 |
MMSE (score) | 29 (26–30) | 28 (24–30) | < 0.001 | 29.5 (27–30) | 28 (20–30) | < 0.0001 |
MoCA (score) | 28 (25–30) | 25 (19–30) | < 0.001 | 30 (23–30) | 25 (12–30) | < 0.0001 |