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Table 2 Demographic data

From: Endophenotypical drift in Huntington’s disease: a 5-year follow-up study

  Premanifest HD gene-expansion carriers Motor manifest HD gene-expansion carriers Level of significance
p values
Premanifest HD gene-expansion carriers Motor manifest HD gene-expansion carriers Level of significance
p values
Cohort 2013 Cohort 2018
Number 51 56   30 44  
Gender m/f 30/21 33/23 0.32 17/13 25/19 1.0
Age at examination (years) 36 (20–54) 50 (24–75) < 0.001 40.5 (30–60) 53.5 (29–80) 0.0002
CAG repeat length 42 (39–48) 43 (40–53) < 0.38 41 (39–48) 43 (40–53) 0.0052
Disease Burden scores 234.0 (108.0–437.0)    241.3 (133.0–437.5)   
UHDRS-motor (score) 2 (0–5) 21 (6–51) < 0.001 1 (0–5) 32 (7–91) < 0.0001
TFC (score) 13 (11–13) 10 (4–13) < 0.001 13 (11–13) 8 (0–13) < 0.0001
MMSE (score) 29 (26–30) 28 (24–30) < 0.001 29.5 (27–30) 28 (20–30) < 0.0001
MoCA (score) 28 (25–30) 25 (19–30) < 0.001 30 (23–30) 25 (12–30) < 0.0001
  1. All data is presented as median (range)
  2. Disease Burden score ((CAG–35.5) * age)
  3. HD Huntington’s disease, m/f male/female, TFC total function capacity, MMSE Mini Mental state examination, MoCA Montreal Cognitive Assessment