Table 1.
HTT mutation carriers | Healthy control | |||||||
---|---|---|---|---|---|---|---|---|
PreHD group | P PreHC vs. HC | EMHD group | p EMHD vs. PreHD | p EMHD vs. HC | Combined PreHD & EMHD | p HC vs. PreHD&EMHD | ||
N | 45 | 11 | 56 | 41 | ||||
Age, years (SE) |
31.4 (1.1) |
0.48 | 36.9 (2.9) |
0.04 | 0.25 | 32.5 (1.1) |
0.9 | 32.8 (1.7) |
Male/female | 19/26 | 0.6 | 8/3a | 0.07 | 0.03 | 27/29 | 0.3 | 15/26 |
Education, years (SE) | 14.4 (0.2) |
0.13 | 14.3 (0.4) |
0.9 | 0.25 | 14.4 (0.2) |
0.1 | 14.8 (0.2) |
CAG repeats (SE) |
43.26 (0.5) |
46.8b (1.5) |
0.003 | 43.9 (0.5) |
− | |||
DBS (SE) |
231.1 (12.7) |
386.4b (37.9) |
<0.001 | 261.6 (15.0) |
− | |||
FAS, words (SE) |
46.5 (2.0) |
0.07 | 33.1a,b (2.6) |
0.004 | <0.001 | 44.0a (1.9) |
0.006 | 51.5 (1.8) |
LMWT (SE) |
5.8 (0.3) |
0.23 | 4.7a,b (0.4) |
0.06 | 0.006 | 5.6 (0.2) |
0.06 | 6.2 (0.2) |
LMWT total score (SE) | 38.6a (1.3) |
<0.001 | 33.4 a,b (1.9) |
0.03 | <0.001 | 37.1a (1.1) |
<0.001 | 44.2 0.6 |
Pictures total score (SE) | 35.4a (0.6) |
0.003 | 29.4a,b (2.2) |
<0.001 | <0.001 | 34.5a (0.6) |
<0.001 | 38.0 (0.6) |
MMSE (SE) |
28.8 (0.6) |
0.07 | 27.1a,b (0.8) |
0.10 | <0.001 | 28.2a (0.5) |
0.008 | 29. (0.1) |
STAI state score (SE) |
38.8 (1.4) |
0.09 | 35.6 (3.4) |
0.33 | 0.97 | 38.2 (1.3) |
0.16 | 35.5 (1.4) |
STAI trait score (SE) |
41.8 (1.7) |
0.42 | 38.2 (4.1) |
0.37 | 0.6 | 41.1 (1.6) |
0.62 | 40.0 (1.4) |
UHDRS-TMS (SE) |
1.3 (0.5) |
0.011 | 18.5a,b (2.7) |
<0.001 | <0.001 | 8.4a (2.4) |
0 | |
SDMT (SE) |
45.6a (3.1) |
<0.001 | 27.6a,b (2.9) |
0.0005 | <0.001 | 38.6a (2.7) |
<0.001 | 59.4 1.6 |
Stroop color naming (SE) | 75.1 (3.7) |
0.22 | 56.2a,b (3.4) |
0.001 | <0.001 | 67.4a (3.1) |
0.003 | 80.3 (2.1) |
Stroop word reading (SE) | 86.5 (3.2) |
0.14 | 70.3a,b (4.8) |
0.007 | <0.001 | 80.3a (3.0) |
0.005 | 92.4 (2.3) |
Stroop interference (SE) | 42.8 (2.4) |
0.63 | 34.9a,b (2.3) |
0.03 | 0.015 | 39.7 (1.8) |
0.11 | 44.5 (2.5) |
TFC (SE) |
13.0 0.00 |
12.2a,b (0.3) |
0.001 | <0.0001 | 12.7a (0.1) |
0.04 | 13.0 0.00 |
Data are presented as the means and standard errors (SE). MMSE, Mini-Mental State Examination; LMWT, Luria Memory Words Test; FAS, Controlled Oral Word Association Test; SST, STAI, Spielberger State–Trait Anxiety Inventory, SDMT, Symbol Digit Modalities Test; TFC – total functional capacity.
significant differences p < 0.05 between healthy control and HTT mutation carriers.
significant differences p < 0.05 between PreHD individuals and EMHD patients.