| |
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
