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Table 2 Initial Assessment Scores and Mean Annual Rate of Change in NIS and NIS-LL Total Scores from Initial Assessment to Each Follow-up Assessment in Eligible Studies

From: Rate of neuropathic progression in hereditary transthyretin amyloidosis with polyneuropathy and other peripheral neuropathies: a systematic review and meta-analysis

First Author/Principal Investigator

Year

Disease Type

Measure

Initial Assessment Scores

Rate of Change from Initial Assessment

N

Mean

SD

Time Point

N

Mean

SE

Bensona [6]

2018

ATTRv-PN

NIS

59

43.40

24.66

35 weeks

56

7.98

1.32

66 weeks

52

18.65

1.76

NIS-LL

59

28.72

15.99

35 weeks

56

3.66

0.76

66 weeks

52

9.61

1.00

Berk [7]

2013

ATTRv-PN

NIS

66

45.40

46.30

1 year

37

10.10

1.63

2 years

28

23.20

2.73

NIS-LL

66

27.20

24.50

1 year

37

6.00

1.10

2 years

28

12.10

1.63

Coelho [8]

2012

ATTRv-PN

NIS-LL

61

11.40

13.54

6 months

57

2.05

0.66

12 months

50

4.72

0.77

18 months

47

5.83

0.92

Hernandez-Ojedab [28]

2012

DPN

NIS

25

7.40

4.70

12 weeks

25

0.70

1.15

Hor [29]

2018

DPN

NIS

150

15.80

8.41

6 months

150

−3.30

0.54

12 months

150

−3.90

0.61

Karamc

2020

CMT

NIS

12

59.79

24.22

Annual rate of change from patients with various follow-up durations

12

1.94

1.34

NIS-LL

12

35.88

12.83

12

1.89

0.92

DPN

NIS

11

12.82

8.68

11

1.19

0.72

NIS-LL

11

8.27

4.31

11

1.09

0.57

ATTRv-PN

NIS

10

29.15

29.91

10

18.31

4.41

NIS-LL

10

15.95

14.00

10

9.35

1.70

IPN

NIS

12

34.35

22.78

12

0.42

0.99

NIS-LL

12

24.25

14.06

12

0.43

0.39

Luigetti [30]

2018

ATTRv-PN

NIS

18

69.61

49.73

12 months

13

6.50

1.38

24 months

3

8.33

5.04

Mundayat [31]

2018

ATTRv-PN

NIS-LL

167

7.57

7.29

Annual rate of change over 2 years

167

2.94

0.37

Sahenkb [19]

2005

CMT

NIS

4

17.75

9.73

24 weeks

4

1.25

5.20

Shy [32]

2008

CMT

NIS

72

62.50

19.25

Annual rate of change from patients with various follow-up durations

72

1.37

0.38

Tesfaye [33]

2007

DPN

NIS-LL

262

6.95

5.00

1 year

211

0.63

0.23

Windebank [34]

2004

Small fiber peripheral neuropathy

NIS

NA

NA

NA

6 months

18

−0.40

1.15

Ziegler [35]

1999

DPN

NIS

165

14.00

10.40

7 months

124

−4.37

0.83

NIS-LL

165

11.00

7.30

7 months

125

−3.37

0.54

Ziegler [37]

2009

DPN

NIS-LL

286

8.80

7.30

160 days

286

−3.70

0.30

Ziegler [36]

2011

DPN

NIS

224

12.20

7.80

2 years

207

0.12

0.43

4 years

207

0.61

0.46

NIS-LL

224

9.50

5.30

2 years

207

0.03

0.29

4 years

207

0.43

0.31

  1. aInitial assessment scores and change scores from the NEURO-TTR trial were extracted from clinical study report (CSR) corresponding to this study, which was made available to researchers by Akcea Therapeutics, Inc.
  2. bChanges scores and the corresponding SEs were calculated from the scores at the initial assessment and each follow-up assessment
  3. cUnpublished dataset
  4. Abbreviations: CMT Charcot-Marie-Tooth disease, DPN diabetic peripheral neuropathy, ATTRv-PN hereditary transthyretin amyloidosis with polyneuropathy, NA not available, NIS Neuropathy Impairment Score, NIS-LL Neuropathy Impairment Score – Lower Limbs, SD standard deviation, SE standard error