PMA (n=23) | ALS (n=30) | HC (n=24) | p Value | p Value | Effect size (Hedge's g) | ||
PMA vs HC | ALS vs HC | PMA | ALS | ||||
MMSE | 28.1 (1.5) | 28.3 (1.5) | 29.0 (1.0) | 0.04 | 0.09 | −0.70 | −0.53 |
Attention/working memory | |||||||
Digits forward raw score | 7.4 (2.0) | 7.7 (2.4) | 8.4 (2.2) | 0.11 | 0.24 | −0.47 | −0.30 |
Digits backward raw score | 4.4 (1.9)* | 5.2 (1.7) | 5.8 (1.7) | 0.01 | 0.22 | −0.76 | −0.35 |
Number–letter sequencing score | 9.4 (2.6) | 9.1 (2.9) | 10.3 (1.5) | 0.19 | 0.10 | −0.42 | −0.49 |
Span | 5.1 (1.1) | 4.9 (1.1) | 5.5 (0.7) | 0.12 | 0.04 | −0.43 | −0.62 |
Executive functions | |||||||
Stroop C word interference on colour naming, time (s)* | 107.1 (35.6) | 121.4 (57.8) | 103.3 (31.7) | 0.70 | 0.18 | −0.11 | −0.37 |
Stroop interference time (Stroop C–Stroop B) (s) | 52.0 (25.0) | 52.7 (40.2) | 47.8 (27.4) | 0.60 | 0.62 | −0.16 | −0.14 |
WCST categories | 5.6 (1.1) | 5.3 (1.2) | 5.5 (0.8) | 0.51 | 0.66 | 0.10 | −0.19 |
Perseverative errors | 7.3 (6.5) | 8.2 (6.3) | 6.8 (5.9) | 0.66 | 0.42 | −0.08 | −0.23 |
Non-pers. errors | 2.1 (3.3) | 3.0 (4.1) | 1.9 (2.0) | 0.60 | 0.86 | −0.07 | −0.32 |
Fluency | |||||||
Category motor speed corr. (s) | 2.0 (1.1) | 2.0 (1.0) | 1.6 (0.5) | 0.04 | 0.04 | −0.46 | −0.48 |
Letter motor speed corr. (s) | 4.1 (1.9) | 4.3 (1.8) | 3.4 (0.9) | 0.19 | 0.04 | −0.47 | −0.60 |
Psychomotor speed | |||||||
Stroop A time (s)* | 43.3 (7.8) | 54.2 (25.5) | 44.7 (5.9) | 0.50 | 0.12 | 0.20 | −0.50 |
Stroop B time (s)* | 55.1 (14.2) | 68.7 (27.0) | 55.4 (8.7) | 0.93 | 0.04 | 0.03 | −0.62 |
Memory | |||||||
Doors A (visual recognition) | 10.3 (2.1) | 9.6 (1.6) | 9.8 (1.8) | 0.32 | 0.80 | 0.25 | −0.12 |
Doors B (visual recognition) | 6.1 (2.0) | 6.0 (2.6) | 7.4 (2.4) | 0.06 | 0.05 | −0.58 | −0.55 |
15 words test direct recall | 39.0 (12.6) | 43.1 (10.9) | 43.3 (7.7) | 0.16 | 0.95 | −0.41 | −0.02 |
15 words test delayed recall 20' | 7.4 (3.8) | 9.0 (3.5) | 8.6 (2.7) | 0.23 | 0.64 | −0.36 | 0.12 |
15 words test recognition | 13.8 (1.2) | 13.8 (1.7) | 14.0 (1.4) | 0.51 | 0.64 | −0.15 | −0.12 |
RBMT story immediate recall | 16.6 (5.5) | 16.3 (6.6) | 16.7 (4.6) | 0.93 | 0.82 | −0.02 | −0.07 |
RBMT story delayed recall | 12.2 (5.0) | 13.1 (6.3) | 12.9 (4.5) | 0.63 | 0.91 | −0.14 | 0.04 |
Language | |||||||
Boston naming test | 54.3 (5.9) | 53.7 (5.5) | 56.6 (1.6) | 0.07 | 0.02 | −0.53 | −0.67 |
Visuospatial functions | |||||||
JOLO | 26.2 (4.4) | 24.8 (4.5) | 26.1 (4.0) | 0.97 | 0.24 | 0.02 | −0.30 |
DAT space relations† | 27.7 (18.2) | 24.7 (20.1) | 25.1 (17.7) | 0.65 | 0.93 | 0.14 | −0.02 |
Mental rotation‡ hand (ms) | 1937 (1017) | 1808 (625) | 1897 (936) | 0.90 | 0.71 | −0.04 | 0.10 |
Mental rotation‡ letter R (ms) | 932 (298) | 910 (329) | 841 (193) | 0.26 | 0.41 | −0.37 | −0.25 |
Values are mean (SD). One sided p values of <0.05 are considered significant (two group t test), shown in bold. Negative effect sizes reflect impaired performance compared with controls.
↵* Speed dependent tests, not motor speed corrected.
↵† n=19, 26 and 22 in PMA, ALS and HC, respectively.
↵‡ n=16, 23 and 21 in PMA, ALS and HC, respectively.
ALS, amyotrophic lateral sclerosis; DAT, Differential Aptitude Test; Fluency motor speed corr, motor speed corrected; HC, healthy controls; JOLO, Judgement of Line Orientation; MMSE, Mini-Mental State Examination; MWCST, Modified Wisconsin Card Sorting Test; PMA, progressive spinal muscular atrophy; RBMT, Rivermead Behavioural Memory Test.