Table 2 Neuropsychiatric symptoms and neuropsychological test performances in the premanifest and the motor manifest gene-expansion carriers

Neuropsychiatric scales

SCL-90-R ^a

  Global Severity Index

49.6 (10.1)

53.6(10.2)

0.041

  Somatization

50.5 (9.7)

50.5 (9.9)

0.979

  Obsessive-compulsive

49.5 (10.3)

57.25 (10.2)

<0.001

  Interpersonal Sensitivity

47.9 (10.5)

51.2 (12.0)

0.134

  Depression

49.3 (10.5)

54.0 (9.0)

0.014

  Anxiety

50.1 (10.9)

53.0 (9.46)

0.141

  Anger-hostility

53.3 (11.6)

52.8 (12.7)

0.848

  Phobic anxiety

50.7 (11.1)

55.1 (12.8)

0.059

  Paranoid ideation

47.4 (9.7)

47.2 (9.6)

0.915

  Psychoticism

47.8 (9.2)

53.4 (10.9)

0.005

  Positive Symptoms Total

49.9 (10.0)

52.6 (9.1)

0.142

  Positive Symptoms Distress Index

52.6 (10.3)

55.7 (10.8)

0.141

HAM-17 ^b

4.5 (4.6)

5.8 (3.7)

0.103

Neuropsychological tests

Trail Making Test A (seconds)

22.8 (5.2)

45.3 (21.2)

<0.001

Trail Making Test B (seconds)

62.2 (16.7)

131.8 (55.5)

<0.001

Symbol Digit Modalities Test (number correct)

49.6 (8.1)

28.3 (9.4)

<0.001

Stroop interference (seconds)

119.0 (21.0)

193.5 (77.3)

<0.001

Category verbal fluency

24.1 (4.2)

16.6 (6.4)

<0.001

Lexical fluency, s-words

14.5 (3.9)

10.7 (5.1)

<0.001

Lexical fluency, a-words

9.4 (3.9)

5.7 (3.1)

<0.001

Reys Complex Figure Test, recall

24.3 (6.3)

18.3 (6.0)

<0.001

Reys Complex Figure Test, copy

35.6 (1.0)

34.0 (2.4)

<0.001

Block design (number correct)

11.9 (0.4)

10.8 (1.8)

<0.001

Raven’s Advanced Progressive Matrices

9.5 (1.7)

6.5 (2.6)

<0.001

Selective Reminding Test, immediate recall (number of errors)

12.49 (7.9)

28.17 (11.6)

<0.001

Selective Reminding Test, delayed recall

7.57 (1.7)

6.02 (2.1)

<0.001