Table 4 Sociodemographic and clinical information for Huntington’s disease gene-carriers at baseline.

From: Cognitive engagement may slow clinical progression and brain atrophy in Huntington’s disease

 

Premanifest

Manifest

HD combined cohort

N

21

24

45

Sex (males/females)

3/18

11/13

14/31

Age (years)

35.71 ± 8.8 (25, 59)

50.21 ± 9.7 (35, 69)

43.44 ± 11.8 (25, 69)

Education

13.33 ± 4.0 (8, 19)

12.08 ± 4.0 (6, 18)

12.67 ± 4.0 (6, 19)

CAP

76.25 ± 16.4 (47.37, 122.01)

107.44 ± 17.6 (73.68, 139.39)

92.89 ± 23.1 (47.37, 139.39)

Estimated age of onset*

47.02 + 8.8 (32.59, 62.64)

–

–

Estimated years to onset†

11.31 ± 8.3 (-8.67, 30.06)

–

–

CRQ

14.38 ± 4.0 (6, 21)

10.88 ± 3.9 (4, 18)

12.51 ± 4.3 (4, 21)

UHDRS-motor

1.30 ± 2.6 (0, 10)

N = 20

21.17 ± 12.7 (4, 58)

12.14 ± 13.8 (0, 58)

N = 44

UHDRS-cogscore

134.40 ± 29.8 (100, 200)

N = 20

77.96 ± 26.9 (21, 117)

N = 23

104.21 ± 39.9 (21, 200)

N = 43

 F-A-S test

 39.30 ± 14.2 (17, 69)

 24.09 ± 9.7 (6, 46)

 31.12 ± 14.1 (6, 69)

 Symbol Digit Modalities

 47.55 ± 9.3 (31, 64)

 28.17 ± 11.1 (11, 50)

 37.19 ± 14.1 (11, 64)

 Stroop interference

 47.55 ± 13.1 (28, 77)

 25.70 ± 10.8 (4, 47)

 35.86 ± 16.1 (4, 77)

PBA-s

21.00 ± 25.6 (0, 87)

18.75 ± 16.0 (1, 67)

19.80 ± 20.8 (0, 87)

 Mood

 8.52 ± 9.7 (0, 28)

 4.71 ± 7.9 (0, 34)

 6.64 ± 8.9 (0, 34)

 Apathy

 3.29 ± 5.5 (0, 16)

 5.63 ± 5.2 (0, 16)

 4.53 ± 5.4 (0, 16)

 Irritability/aggression

 3.10 ± 6.4 (0, 25)

 2.71 ± 4.3 (0, 16)

 2.89 ± 5.4 (0, 25)

 Psychosis

 1.33 ± 3.7 (0, 16)

 0.00 ± 0.0 (0, 0)

 0.62 ± 2.6 (0, 16)

 Dysexecutive behaviors

 4.76 ± 8.0 (0, 28)

 4.67 ± 4.4 (0, 18)

 4.71 ± 6.3 (0, 28)

  1. Data presented as mean ± standard deviation (minimum, maximum). N(number of participants) detailed in individual cells when differing. Premanifest and manifest categories designated based on Unified Huntington’s Disease Rating Scale diagnostic confidence score for motor abnormalities at first visit101.
  2. * Estimated age of onset based on model by Langbehn et al. (2010)15.
  3. † Estimated years to onset calculated as difference between estimated age of onset and current age.
  4. CAP=standardized CAG-age product87; CRQ=Cognitive Reserve Questionnaire91; HD = Huntington’s disease; N=number of participants; UHDRS-cogscore=Unified Huntington’s Disease Rating Scale total cognitive score102; UHDRS-motor=Unified Huntington’s Disease Rating Scale total motor score; PBA-s=short-Problem Behavior Assessment89.
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