Table 3.
Neuropsychological test performance of study participants
| Neuropsychological measures | Fabry disease |
Controls |
||||||
|---|---|---|---|---|---|---|---|---|
| Males |
Females |
Males |
Females |
|||||
| M (SD) | Range | M (SD) | Range | M (SD) | Range | M (SD) | Range | |
| Premorbid | ||||||||
| Test of premorbid functioninga | 102 (9.4) | 79–122 | 104 (15.5) | 88–125 | 109.3 (16.03) | 82–126 | 107.8 (14.9) | 84–124 |
| General intellectual | ||||||||
| Full scale IQa | 94.9 (13.1) | 75–121 | 109 (15.3) | 94–130 | 113 (10.9) | 98–135 | 116.8 (10.4) | 100–129 |
| Verbal comprehension (VCI)a | 97.7 (14.8) | 70–134 | 107.6 (13.8) | 95–125 | 115.8 (12.2) | 95–130 | 110.5 (11.9) | 87–118 |
| Perceptual reasoning (PRIa) | 97.3 (11.6) | 82–115 | 106 (15.2) | 88–127 | 111.3 (13.4) | 92–142 | 121.3 (10.3) | 107–131 |
| Information processing | ||||||||
| Processing speed (PSI)a | 90.9 (14.5) | 65–122 | 110.2 (15.3) | 94–134 | 103.9 (11.1) | 86–120 | 108.2 (6.01) | 100–114 |
| Immediate attention | ||||||||
| Digits forwardb | 9.2 (1.8) | 7–12 | 10.4 (3.2) | 6–14 | 10.9 (3.3) | 7–18 | 10.3 (3.01) | 7–16 |
| CVLT-II first learning trialc | −0.5 (1.4) | −2.5–2.5 | −0.1 (1.02) | −1–1.5 | −0.27 (0.9) | −1.5–1 | 0.42 (1.2) | −1.0–2.0 |
| Memory | ||||||||
| Auditory Memory Indexa | 104 (13.9) | 84–131 | 111.2 (14.8) | 91–130 | 108.2 (10.7) | 88–124 | 116.2 (9.4) | 101–128 |
| Visual Memory Indexa | 98.3 (16.8) | 73–134 | 93.8 (4.7) | 86–98 | 106.2 (10.4) | 93–121 | 109.2 (9.9) | 98–125 |
| Immediate Memory Indexa | 100.7 (18.3) | 67–133 | 103 (8.6) | 94–115 | 108.6 (9.3) | 96–125 | 114 (7.1) | 107–123 |
| Delayed Memory Indexa | 101.9 (15.9) | 82–137 | 103.2 (8.9) | 92–114 | 109.7 (9.5) | 100–126 | 114.5 (10.3) | 100–128 |
| CVLT-II total verbal learningc | 0.5 (1.5) | −1.1–2.8 | 1 (0.5) | 0.4–1.7 | 1 (1) | −0.3–2.4 | 0.3 (0.6) | −0.3–1.2 |
| CVLT-II short delay free recallc | 0.1 (0.9) | −1–2 | 0.6 (0.4) | 0–1 | 0.6 (0.5) | 0–1.5 | 0.9 (0.8) | 0–2.0 |
| CVLT-II short delay cued recallc | 0.3 (0.7) | −1.5–1.5 | 0.3 (1.2) | −1.5–1.5 | 0.5 (0.6) | −0.5–1.5 | 0.6 (0.9) | −1.05–1.5 |
| CVLT-II long delay free recallc | 0.3 (0.7) | −1–1.5 | 0.4 (0.9) | −1–1.5 | 0.5 (0.6) | 0–1.5 | 0.6 (0.9) | −0.5–1.5 |
| CVLT-II long delay cued recallc | 0.2 (0.8) | −1–1.5 | 0.4 (0.8) | −0.5–1.5 | 0.3 (0.7) | −1–1.5 | 0.7 (0.9) | −1.0–1.5 |
| CVLT-II recognition hitsd | 15 (1.2) | 12–16 | 14.8 (1.3) | 13–16 | 14.9 (1.2) | 13–16 | 15.8 (0.4) | 15–16 |
| CVLT-II recognition false positivesd | 2.3 (3.1) | 0–10 | 0.2 (0.4) | 0–1 | 0.8 (1.1) | 0–3 | 0.2 (0.4) | 0–1 |
| Language | ||||||||
| Boston Naming Testd | 57.5 (2.6) | 52–60 | 58.6 (3.1) | 53–60 | 59 (1.3) | 57–60 | 59 (1.6) | 56–60 |
| WAIS-IV vocabularyb | 10.2 (3.5) | 4–19 | 11.6 (2.8) | 9–16 | 13.2 (2.6) | 8–16 | 12.7 (3.2) | 7–16 |
| Visuospatial | ||||||||
| WAIS-IV block designb | 10.6 (2.5) | 6–14 | 11.2 (3.3) | 8–15 | 11.8 (3.1) | 9–19 | 13.5 (1.7) | 11–16 |
| Working memory | ||||||||
| Digits backwardb | 8.7 (1.6) | 7–13 | 12 (3.5) | 9–18 | 10.1 (2.9) | 6–15 | 10.2 (2.4) | 8–14 |
| Digits sequencingb | 9.7 (3.1) | 5–15 | 12 (3.5) | 7–12 | 12.2 (2.3) | 9–17 | 11.5 (0.8) | 11–13 |
| Reasoning skills (executive) | ||||||||
| WAIS-IV similaritiesb | 9 (2.1) | 6–14 | 11 (2.8) | 9–15 | 11 (2.4) | 8–15 | 10.8 (1.9) | 8–13 |
| WAIS-IV matrix reasoningb | 9.2 (2.2) | 6–12 | 10.2 (2.7) | 7–14 | 11.9 (2.7) | 8–16 | 13.5 (2.8) | 10–17 |
| WAIS-IV visual puzzlesb | 9.3 (2.6) | 6–13 | 11.8 (2.6) | 9–15 | 12.4 (3.6) | 9–19 | 14.7 (1.2) | 13–16 |
| Verbal fluency (executive) | ||||||||
| Letter fluencyc | −1 (0.9) | −2.1–1.5 | 0.2 (0.6) | −0.7–0.7 | 0.2 (0.4) | −0.3–0.9 | 0.5 (0.9) | −0.5–1.9 |
| Semantic fluencyc | −0.7 (1.1) | −2.8–0.5 | 0.1 (0.8) | −0.9–1.4 | 0.8 (0.6) | 0.05–1.9 | 0.9 (0.9) | −4.2–7.9 |
| Problem solving/perseveration (executive) | ||||||||
| WCST categoriesd | 5.25 (1.2) | 3–6 | 6 (0) | 6 | 6 (0) | 6 | 6 (0) | 6 |
| WCST perseverative errorsa | 98 (19.5) | 76–129 | 103.6 (6.8) | 97–115 | 122.67 (14.4) | 104–145 | 110.5 (17.5) | 95–140 |
Notes: CVLT-II = California Verbal Learning Test, Second Edition; WAIS-IV = Wechsler Adult Intelligence Scale, Fourth Edition; WCST = Wisconsin Card Sorting Test.
aStandard scores (M = 100, SD = 15).
bScaled scores (M = 10, SD = 3).
cz-scores (M = 0, SD = 1).
dRaw scores.