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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