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Table 1 Laboratory and clinical data at baseline and six months (mean ± standard deviation) and statistical significance results

From: A feasibility study of mHealth and wearable technology in late onset GM2 gangliosidosis (Tay-Sachs and Sandhoff Disease)

 

Baseline

Month 6

Wilcoxon p-value

6MWT (meters)

316.88 ± 123.26

345.50 ± 117.68

0.11

BARS score

9.75 ± 6.09

10.06 ± 6.96

0.59

Neuroglyphics Off Target-dominant (%)

19.00 ± 14.11

16.62 ± 10.91

0.69

Neuroglyphics Off Target- Nd (%)

21.19 ± 13.99

17.77 ± 11.65

0.47

BARS Upper score

2.63 ± 2.22

2.75 ± 2.84

0.79

9HP Dom Avg (sec)

28.83 ± 8.23

27.22 ± 7.84

0.38

9HP Dom z-score (sec)

4.35 ± 2.77

4.04 ± 2.77

0.38

9HP Nd Avg (sec)

30.89 ± 15.88

29.84 ± 11.41

0.78

9HP Nd z-score (sec)

4.71 ± 5.39

5.05 ± 4.96

1

GAITRite data:

Cadence (steps/min)

94.33 ± 18.86

99.95 ± 13.88

0.15

Velocity (cm/sec)

97.86 ± 34.08

108.59 ± 33.61

0.15

Step Length (cm)

60.10 ± 12.74

63.68 ± 14.62

0.38

Step Width (cm)

11.72 ± 3.77

11.53 ± 3.46

0.55

Step Time (sec)

0.67 ± 0.15

0.61 ± 0.09

0.11

  1. Avg Average, BARS Brief Ataxia Rating Scale, cm Centimetres, DOM Dominate min Minute, 6MWT 6-min walk test, Nd Non-dominant, sec Seconds, 9HP 9-hole peg test – a brief, standardized and quantitative test of upper extremity (hand and arm) function z-score: calculated by converting raw into a common metric