Table 2.
Demographic information per group
| Manifest HD | Pre-HD | |||||||
|---|---|---|---|---|---|---|---|---|
| FA | TFC | TFC | ||||||
| FAunimpaired (n = 1179) | FAimpaired (n = 339) |
TFCunimpaired (n = 380) |
TFCmild (n = 233) |
TFCmoderate (n = 209) |
TFCsevere (n = 90) |
PreHDcontrol (n = 118) |
PreHDimpaired (n = 31) |
|
| Age (years) | 52 (11.7) | 54.2 (12)* | 51.9 (11.4) | 50.6 (11.5) | 52.7 (11.7) | 53.4 (11.5) | 41.4 (12.1) | 37.3 (10.8) |
| CAG repeat length | 43.3 (2.9) | 44 (3.3)*** | 42.8 (2.5) | 43.8 (3)*** | 44 (3.4)*** | 43.9 (3.3)*** | 42 (2.4) | 43.8 (3.2)*** |
| DBS | 378 (77.8) | 441.4 (146.6)*** | 353.9 (72.5) | 406.8 (142.8)*** | 416.8 (93.2) *** | 422.8 (98.7)*** | 250 (69.7) | 288 (83)* |
| ISCED | 3.6 (1.2) | 3.4 (1.2)* | 3.7 (1.2) | 3.5 (1.3) | 3.6 (1.2)* | 3 (1.2)*** | 4.2 (1.1) | 3.8 (1) |
| Job class [% unemployed] | 3.1 (1.3) [66.4%] | 3.8 (0.7)*** [91.7%] | 2.6 (1.4) [47.6%] |
2.9 (1.3)* [58.8%] |
3.6 (0.9)*** [85.2%] |
3.9 (0.5)*** [92.8%] |
1.8 (1.1) | 2.3 (1.4)* |
| Sex (% female) | 50.2% | 55.2% | 49.5% | 44.6% | 50.7% | 42.7% | 65.7% | 74.2% |
| UHDRS motor score | 26.3 (13.6) | 47 (16.4)*** | 19.6 (10) | 28.3 (12.9)*** †¥ | 37.1 (14.5) ***‡ | 43.2 (16.9)*** | 1.1 (1.4) | 2.3 (2)*** |
| SDMT | 30.4 (11.4) | 16 (8.5)*** | 35.6 (10.9) | 27.3 (10.9)*** †¥ | 21.1 (9.7)*** † | 15.3 (8.2)*** | 54 (9.4) | 46.2 (10.5)*** |
| Verbal Fluency | 15.1 (5) | 9.8 (4.3)*** | 16.9 (4.9) | 14.1 (5)*** †¥ | 11.7 (4.7)*** † | 9.3 (4.4)*** | 22.3 (5.8) | 20.6 (5.6) |
| Stroop naming | 50.9 (14.7) | 32.9 (11.8)*** | 57.9 (13.7) | 47 (14.1)*** †¥ | 40.3 (12.4)*** † | 31.3 (11.6)*** | 75.9 (14.1) | 73.7 (15.9) |
| Stroop word | 67.2 (19.3) | 44.2 (15.7)*** | 76.4 (17.2) | 62.6 (19.6)*** †¥ | 54.4 (17.5)*** † | 40.9 (15.2)*** | 97.7 (16.4) | 87.8 (18.1)* |
| Stroop interference | 29 (10.4) | 17 (8.6)*** | 33.6 (9.6) | 26.7 (10.1)*** †¥ | 20.9 (7.9)*** † | 16.5 (8.8)*** | 44.3 (8.9) | 42.4 (9.7) |
| Trail A score | 24.9 (1.7) | 24.1 (4.1) | 24.9 (1.9) | 24.9 (1.7) ‡ | 24.8 (0.2) † | 24 (4.3)*** | 25 (0) | 25 (0) |
| Trail A time | 52.5 (30.2) | 95.5 (53.6)*** | 46.9 (25.7) | 46.9 (24.7) †¥ | 72.5 (38.6)*** † | 107.8 (59.9)*** | 25.1 (8.9) | 28.1 (10.6) |
| Trail B score | 23.7 (4.3) | 17.8 (8.6)*** | 23.9 (3.8) | 24.1 (3.5) † | 20.8 (7.1)*** † | 16.9 (8.9)*** | 25 (0) | 25 (0) |
| Trail B time | 123.2 (64.5) | 193.9 (59.8)*** | 109 (62) | 111.2 (60.8) | 171.2 (65.8)*** | 204 (61.2)*** | 50.3 (20) | 57.5 (24.9) |
| PBA Irritability | 3.1 (4.6) | 3.3 (5.1) | 2.4 (3.8) | 2.9 (4.1) | 3.5 (4.8)* | 3.1 (4.9) | 1.1 (2.2) | 4.5 (5.7)*** |
| PBA Apathy | 2.5 (3.7) | 4.7 (4.7)*** | 1.6 (3) | 2.4 (3.5)*** †¢ | 3.4 (3.9)*** ‡ | 4.8 (4.6)*** | 0.7 (2) | 2 (3.2)* |
| PBA Depression | 5.1 (6.3) | 5.5 (6.9) | 4.5 (6) | 4.9 (5.3) | 5.8 (6.2)* | 5.4 (6) | 3.2 (5.3) | 9 (8.3)*** |
| MMSE | 26.9 (2.6) | 23.7 (3.9)*** | 27.9 (1.9) | 26.7 (2.6) †¥ | 25.4 (2.9)*** | 23.3 (3.6)*** | 28.9 (1.3) | 28 (2)* |
T tests were used for the FA and Pre-HD analysis and an ANOVA was used for the TFC analysis. Shows mean and standard deviation in brackets
DBS disease burden score, MMSE mini-mental state examination, SDMT symbol digit modality, PBA problem behaviour assessment (PBA) – short version; ISCED International standard classification of education
*Indicates a significant difference (p < 0.05) when compared with controls for each group
***Indicates a significant difference (p < 0.001) when compared with controls for each group
†Indicates a significant difference (p < 0.001) when compared with severely impaired patients
‡Indicates a significant difference (p < 0.05) when compared with severely impaired patients
¥Indicates a significant difference (p < 0.001) when compared with moderately impaired patients
¢Indicates a significant difference (p < 0.05) when compared with moderately impaired patients