Table 4.
Comparison of cognitive performance between SCA14 affected subjects and intrafamilial controls
| |
Norm data |
Affectedn = 9 |
Intrafamilial controlsn = 10 |
Affected vs norm |
Controls vs norm |
Affected vs controls |
Subject, V-10** |
SubjectIII 3*** |
|---|---|---|---|---|---|---|---|---|
| |
|
|
|
One-sample t-test
|
One-sample t-test
|
MannWhitney U test
|
|
|
| Mean | Median ± interquartile range | Median ± interquartile range | p -value* | p-value* | p -value* | Scores | Scores | |
| General cognitive functioning |
|
|
|
|
|
|
|
|
| Intelligence Quotient (IQ) |
100 |
92.0 ± 10 |
100.5 ± 18 |
0.020 |
0.563 |
0.288 |
91 |
92 |
| Vocabulary (t-score) |
50 |
41.0 ± 8 |
44.0 ± 14 |
0.001 |
0.194 |
0.120 |
43 |
39 |
| Matrix Reasoning (t-score) |
50 |
53.0 ± 11 |
51.5 ± 18 |
0.479 |
0.602 |
1.000 |
47 |
52 |
| Verbal executive function |
|
|
|
|
|
|
|
|
| Controlled Word Fluency Test, Phonologic (FAS) t-score |
50 |
45.0 ± 6 |
46.5 ± 9 |
0.003 |
0.174 |
0.410 |
43 |
40 |
| Controlled Word Fluency Test, Semantic t-score |
50 |
53.0 ± 9 |
53.0 ± 9 |
0.506 |
0.200 |
0.711 |
47 |
55 |
| Learning and memory |
|
|
|
|
|
|
|
|
| CVLT Learning percentil |
50 |
59.0 ± 11 |
48.0 ± 9 |
0.866 |
0.866 |
0.041 |
52 |
65 |
| CVLT Long-Delay Free Recall |
10 |
14.0 ± 2 |
12.0 ± 2 |
<0.001 |
0.005 |
0.065 |
9 |
12 |
| CVMT Learning percentil |
50 |
10.3 ± 42.2 |
11.5 ± 19.3 |
0.022 |
<0.001 |
0.806 |
8.6 |
10.3 |
| CVMT Memory percentil |
50 |
27.6 ± 59.6 |
15.1 ± 10.8 |
0.278 |
<0.001 |
0.135 |
13.8 |
27.6 |
| Visuoperceptive skills |
|
|
|
|
|
|
|
|
| Line Orientation Test |
25.4 |
27.0 ± 11 |
28.0 ± 3 |
0.359 |
0.288 |
0.136 |
19 |
17 |
| Working memory |
|
|
|
|
|
|
|
|
| Digit Span, sub-test, WAIS-R |
10 |
10.0 ± 5 |
10.0 ± 3 |
0.540 |
0.310 |
0.868 |
8 |
10 |
| Paced Auditory Serial Addition Test PASAT |
46 |
39.0 ± 22 |
38.5 ± 10 |
0.049 |
0.036 |
0.653 |
**** |
32 |
| Psychomotor speed |
|
|
|
|
|
|
|
|
| Stroop Color Naming Test 1 |
10 |
7.0 ± 3 |
9.5 ± 5 |
<0.001 |
0.111 |
0.284 |
7 |
6 |
| Stroop Color Naming Test 2 |
10 |
7.0 ± 2 |
7.0 ± 5 |
0.003 |
0.026 |
0.706 |
3 |
7 |
| Executive functions, inhibition and set-shifting |
|
|
|
|
|
|
|
|
| Stroop Color Naming Test 3 |
10 |
9.0 ± 4 |
9.0 ± 3 |
0.373 |
0.327 |
0.868 |
3 |
12 |
| Stroop Color Naming Test 4 |
10 |
9.0 ± 4 |
10.5 ± 3 |
0.111 |
0.842 |
0.137 |
6 |
9 |
| WCST total errors t-score |
50 |
53.0 ± 20 |
38.5 ± 10 |
0.928 |
0.005 |
0.141 |
46 |
56 |
| WCST categories |
5.1 |
6.0 ± 1 |
5.5 ± 2 |
***** |
***** |
0.257 |
5 |
6 |
| WCST conceptual level responses t-score |
50 |
52.0 ± 20 |
39.0 ± 6 |
0.977 |
0.001 |
0.093 |
47 |
58 |
| WCST perseverative responses t-score | 50 | 55.0 ± 26 | 37.0 ± 12 | 0.661 | 0.002 | 0.064 | 49 | 55 |
*p-values < 0.05 are highlighted in bold.
**Subject V-10’s individual scores. V-10 is confirmed to carry SCA14 mutation, but was motor asymptomatic and not included in group analyses.
***Subject III-3’s individual scores. III-3 is the only subject from Family 2, and is also included in affected group analyses.
****Subject V-10 did not fulfill PASAT test.
*****not available for t-test due to not normal distribution.