Table 1.
Group demographics with clinical and behavioral scores
| Healthy controls | preHD (A and B) | preHD-A | preHD-B | Manifest HD | ||
|---|---|---|---|---|---|---|
| N | 24 | 22‡ | 11 | 11 | 10 | |
| Gender male/female | 11/13 | 9/13 | 4/7 | 5/6 | 4/6 | |
| Age in years (at V1), mean (SD) | 49.0 (8.2) | 43.6 (8.7) | 44.2 (5.7) | 43.0 (11.2) | 50.2 (9.3) | |
| Handedness R/L | 20/4 | 18/4 | 9/2 | 9/2 | 9/1 | |
| Level of education (ISCED), median (range) | 4 (3) | 4 (3) | 4 (3) | 4 (3) | 4 (3) | |
| DART-IQ, mean (SD) | 105.0 (9.4) | 100.5 (11.2) | 101.3 (9.7) | 99.6 (13.0) | 101.8 (13.5) | |
| CAG repeat length, mean (SD) | n/a | 42.6 (2.7) | 41.3 (1.4) | 43.9 (3.1)^ | 42.5 (1.2) | |
| Estimated years to onset, mean (SD) | n/a | 11.8 (4.7) | 14.9 (4.7) | 8.6 (1.8)^ | n/a | |
| Total functional capacity, mean (SD) | ||||||
| V1 | 13.0 (0.2) | 12.8 (0.5) | 12.7 (0.7) | 12.8 (0.4) | 11.0 (1.5)Φ | |
| V2 | 12.9 (0.5) | 12.6 (0.9) | 12.7 (0.6) | 12.5 (1.0) | 10.3 (2.2)Φ | |
| UHDRS-TMS, mean (SD) | ||||||
| V1 | 2.6 (2.5) | 2.6 (1.5) | 2.0 (1.5) | 3.1 (1.2) | 14.6 (7.7)Φ | |
| V2 | 2.1 (1.6) | 5.7 (5.1) ¥ | 3.5 (2.2) | 8.3 (6.1)*^ | 23.0 (12.1)Φ | |
| SDMT, mean (SD) | ||||||
| V1 | 49.4 (8.9) | 50.1 (11.0) | 53.5 (9.3) | 46.7 (11.9) | 41.2 (9.2)Φ | |
| V2 | 50.9 (9.3) | 50.6 (10.0) | 54.7 (10.0) | 46.6 (8.5)^ | 39.2 (10.6)Φ | |
| SWR, mean (SD) | ||||||
| V1 | 100.1 (13.2) | 91.9 (14.2)* | 95.6 (9.6) | 88.3 (17.3)* | 87.7 (14.7)* | |
| V2 | 102.0 (15.6) | 87.9 (15.7)* | 91.4 (9.4) | 84.4 (20.0)* | 86.4 (18.6)* | |
| BDI-II, mean (SD) | ||||||
| V1 | 4.1 (4.4) | 6.4 (6.4) | 4.9 (6.0) | 7.9 (6.8) | 10.2 (8.2)* | |
| V2 | 3.9 (4.1) | 5.1 (5.6) | 3.2 (4.9) | 6.9 (5.9) | 8.2 (8.4) | |
| Between-scan interval in months, mean (SD) | 23.0 (0.8) | 23.0 (0.7) | 23.2 (0.6) | 22.7 (0.7) | 23.5 (0.7) | |
N number of participants, SD Standard deviation, n/a not applicable, ISCED International Standard Classification of Education, DART-IQ Dutch Adult Reading Test Intelligence Quotient, CAG Cytosine-Adenine-Guanine, UHDRS-TMS Unified Huntington’s Disease Rating Scale-Total Motor Score, SDMT Symbol Digit Modalities Test, SWR Stroop Word Reading task, BDI-II Beck Depression Inventory-II, V1 visit 1, V2 visit 2
Significance at p ≤ 0.05 level: * significantly different from controls, Φ significantly different from controls and preHD, ¥ significantly different from controls and HD, ^ significantly different from preHD-A
‡ Including five subjects progressing to the early manifest stage during the two year follow-up period