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. Author manuscript; available in PMC: 2016 Sep 9.
Published in final edited form as: Mol Genet Metab. 2016 Apr 25;118(2):111–122. doi: 10.1016/j.ymgme.2016.04.005

Table 2.

ADL scores for age-matched controls, patients with attenuated phenotypes, and patients with severe phenotypes.

Subject age range (years)
0–5 5–10 10–15 15–20 20<
Control n 42 58 26 5 14
Movement 14.57 ± 6.26 19.40 ± 0.56 19.96 ± 0.20 20.00 ± 0.00 20.00 ± 0.00
Movement Cognition 8.79 ± 6.54 19.19 ± 1.89 19.88 ± 0.43 20.00 ± 0.00 20.00 ± 0.00
Cognition 8.38 ± 5.49 15.93 ± 2.55 18.81 ± 1.83 20.00 ± 0.00 20.00 ± 0.00
Severe n 7 4 11 13 28
Movement 12.43 ± 4.50 14.88 ± 2.66 10.68 ± 4.05 8.46 ± 4.67 8.23 ± 5.32
pa 0.3908 p < 0.0001 p < 0.0001 p < 0.0001 p < 0.0001
Movement with Cognition 11.00 ± 6.76 14.13 ± 4.94 13.77 ± 2.09 12.88 ± 4.10 12.46 ± 5.78
pa 0.4129 p < 0.0001 p < 0.0001 p < 0.005 p < 0.0001
Cognition 14.00 ± 8.10 19.75 ± 0.50 19.27 ± 2.10 19.38 ± 1.19 18.23 ± 2.69
pa p < 0.05 p < 0.005 0.5038 0.2743 p < 0.05
Attenuated n - 3 1 3 10
Movement - 16.33 ± 3.06 18.00 14.50 ± 3.50 13.25 ± 3.58
pa - p < 0.0001 NA p < 0.01 p < 0.0001
Movement with Cognition - 19.67 ± 0.58 18.00 17.33 ± 2.31 17.70 ± 2.06
pa - 0.6661 NA p < 0.05 p < 0.001
Cognition - 20.00 ± 0.00 20.00 20.00 ± 0.00 19.70 ± 0.67
pa - p < 0.01 NA NA 0.1074
t-Test between phenotypes Movement - 0.5289 NA 0.0559 p < 0.01
Movement with Cognition - 0.1177 NA 0.0961 p < 0.01
Cognition - 0.4366 NA 0.3990 0.0986
a

Control vs. severe or attenuated phenotype.

NA: not applicable.