Table 1.
Demographic and clinical baseline characteristics
| Premanifest HD | Controls | |||
|---|---|---|---|---|
| Non-converters | Converters | Combined | ||
| Number of participants | 9 | 20 | 29 | 28 |
| Gender (male/female) | 3/6 | 9/11 | 12/17 | 13/15 |
| Age (years) | 41.6 (6.5) | 44.2 (8.7) | 43.3 (8.0) | 48.6 (7.9) † |
| CAG repeat length | 42.4 (1.8) | 43.8 (2.6) | 43.4 (2.4) | – |
| Disease burden score | 286 (56.9) | 352 (61.3)* | 332 (66.6) | – |
| UHDRS - TMS | 1.9 (1.8) | 2.7 (1.3) | 2.4 (1.5) | 2.6 (2.4) |
Data is given in mean (standard deviation). Premanifest HD gene carriers were divided into non-converters with a Diagnostic Confidence Level (DCL) below 4 and converters who progressed to manifest HD rated as a DCL of 4. CAG: cytosine-adenine-guanine. UHDRS-TMS: Unified Huntington’s Disease Rating Scale – Total Motor Score. Disease burden score = age x (CAG length − 35.5) by Penney et al. [12]
*Significantly different between converters and non-converters at p < 0.05
†Significantly different between controls and premanifest HD combined group at p < 0.05