Table 1

Clinical characteristics of the two groups of participants
Premanifest HD gene-expansion carriers Motor manifest HD gene-expansion carriers Level of significance
N = 51 N = 56 p-values
Gender (m/f)§ 30/21 33/23 p = 0.32
Age at examination#(years) 36 (20–54) 50 (24–75) p < 0.001
CAG repeat length# 42 (39–48) 43 (40–53) p < 0.38
Disease burden scorea 234.0 (108.0–437.0)
UHDRS-motorb(score)# 2 (0–5) 21 (6–51) p < 0.001
TFCc(score)# 13 (11–13) 10 (4–13) p < 0.001
MMSE (score)# 29 (26–30) 28 (24–30) p < 0.001
MoCA (score)# 28 (25–30) 25 (19–30) p < 0.001

Data are presented as median (range).

p values were calculated by §Chi-square-test or #Mann–Whitney U test.

aDisease burden score = ((CAGn – 35.5)*Age); bUHDRS-motor: Unified Huntington’s Disease Rating Scale-99, range (0–124); cTFC: Total Function Capacity, range (0–13).

Vinther-Jensen et al.

Vinther-Jensen et al. Orphanet Journal of Rare Diseases 2014 9:114   doi:10.1186/s13023-014-0114-8

Open Data