Table 2.
Subject characteristics by group. Values presented are mean (SD) or N (%) where indicated. All scores on neuropsychological tests are standard scores which have a population mean of 100 and a standard deviation of 15).
| Variables | Control (N=55) | MPS II (all) (N=20) | MPS II (<25 years of age) (N=16) | MPS II (>25 years of age (N=4) |
|---|---|---|---|---|
| Site: | ||||
| University of Minnesota | 55 (100.0%) | 16 (80.0%) | 14 (87.5%) | 2 (50.0%) |
| Other sites | 0 (0.0%) | 4 (20.0%) | 2 (12.6%) | 2(50.0%) |
|
| ||||
| Age at evaluation | 12.8 (5.2) | 15.8 (13.2) | 9.6 (3.2) | 40.5 (5.7) |
|
| ||||
| Age at diagnosis | NA | 5.9 (3.9) | 5.1 (2.9) | 9.3 (6.0) |
|
| ||||
| Age at ERT start | NA | 12 (12.4) | 6.3 (3.3) | 35 (5.6) |
|
| ||||
| Time since first ERT | NA | 3.6 (2.2) | 3.2 (2.1) | 5.4 (1.7) |
|
| ||||
| Test Results: | ||||
| IQ | 112 (13.24) | 99.25 (17.85) | 98.00 (18.47) | 104.25 (16.46) |
| Memory | ||||
| Verbal Memory (M=100, SD 15) | NA | 98.16 (18.84) | 98.64 (16.26) | 87.29 (19.35) |
| Story Memory (M=100, SD 15) | NA | 107 (19.81) | 105.02 (18.91) | 108.74 (17.52) |
| Visual Memory (M=100, SD 15) | NA | 86.95 (17.83) | 85.53 (18.84) | 92.28 (14.30) |
| Visual Motor (M=100, SD 15) | NA | 79.59 (24.83) | 76.07 (25.21) | 91.90 (21.91) |
| Spatial Ability (M=100, SD 15) | NA | 88.54 (17.98) | 86.35 (19.71) | 96.78 (3.26) |
| Attention | ||||
| TOVA/CPT Omission errors (M=100, SD 15) | 92.71 (7.17) | 84.69 (23.04) | 78.07 (27.76) | 85.50 (20.98) |
| TOVA/CPT Commission errors (M=100, SD 15) | 98.23 (17.85) | 97.38 (18.86) | 90.29 (19.17) | 113.50 (10.02) |
| TOVA/CPT Variability (VAR) (M=100, SD 15) | 94.61 (18.48) | 81.44 (27.85) | 76.71 (26.57) | 94.50 (32.58) |
| TOVA/CPT Reaction Time (RT) (M=100, SD 15) | 95.73 (15.55) | 88.88 (25.29) | 88.79 (25.53) | 91.25 (22.95) |
| Executive Function | ||||
| SOC: Problems solved (M=100, SD 15) | NA | 94.20 (20.94) | 93.56 (22.00) | 98.05 (18.46) |
| SWM: Errors (M=100, SD 15) | NA | 100.75 (21.08) | 99.09 (22.42) | 110.73 (4.14) |
|
| ||||
| PSS: Physical Symptom Scale | NA | 8.30 (1.87) | 7.88 (1.59) | 10.00 (2.16) |
|
| ||||
| Brain Volumes – absolute values | ||||
| Corpus callosum (mL) | 3.02 (0.51) | 2.57 (0.66) | 2.36 (0.51) | 3.37 (0.57) |
| Cortical white matter (mL) | 454 (61.66) | 474 (58.60) | 458 (47.32) | 534 (63.47) |
| Cortical gray matter (mL) | 591 (52.82) | 583 (78.45) | 611 (60.11) | 478 (38.57) |
| Frontal lobes (mL) | 219 (20.24) | 219 (30.51) | 229 (22.50) | 170 (7.71) |