Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial

Peter J. Dyck; John C. Kincaid; P. James B. Dyck; Vinay Chaudhry; Namita A. Goyal; Christina Alves; Hayet Salhi; Janice F. Wiesman; Celine Labeyrie; Jessica Robinson-Papp; Márcio Cardoso; Matilde Laura; Katherine Ruzhansky; Andrea Cortese; Thomas H. Brannagan; Julie Khoury; Sami Khella; Márcia Waddington-Cruz; João Ferreira; Annabel K. Wang; Marcus V. Pinto; Samar S. Ayache; Merrill D. Benson; John L. Berk; Teresa Coelho; Michael Polydefkis; Peter Gorevic; David H. Adams; Violaine Plante-Bordeneuve; Carol Whelan; Giampaolo Merlini; Stephen Heitner; Brian M. Drachman; Isabel Conceição; Christopher J. Klein; Morie A. Gertz; Elizabeth J. Ackermann; Steven G. Hughes; Michelle L. Mauermann; Rito Bergemann; Karen A. Lodermeier; Jenny L. Davies; Rickey E. Carter; William J. Litchy

doi:10.1002/mus.25563

Assessing mNIS+7_Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial

Peter J. Dyck, John C. Kincaid, P. James B. Dyck, Vinay Chaudhry, Namita A. Goyal, Christina Alves, Hayet Salhi, Janice F. Wiesman, Celine Labeyrie, Jessica Robinson-Papp, Márcio Cardoso, Matilde Laura, Katherine Ruzhansky, Andrea Cortese, Thomas H. Brannagan, Julie Khoury, Sami Khella, Márcia Waddington-Cruz, João Ferreira, Annabel K. WangMarcus V. Pinto, Samar S. Ayache, Merrill D. Benson, John L. Berk, Teresa Coelho, Michael Polydefkis, Peter Gorevic, David H. Adams, Violaine Plante-Bordeneuve, Carol Whelan, Giampaolo Merlini, Stephen Heitner, Brian M. Drachman, Isabel Conceição, Christopher J. Klein, Morie A. Gertz, Elizabeth J. Ackermann, Steven G. Hughes, Michelle L. Mauermann, Rito Bergemann, Karen A. Lodermeier, Jenny L. Davies, Rickey E. Carter, William J. Litchy

School of Medicine

Research output: Contribution to journal › Article › peer-review

29 Scopus citations

Abstract

Introduction: Polyneuropathy signs (Neuropathy Impairment Score, NIS), neurophysiologic tests (m+7_Ionis), disability, and health scores were assessed in baseline evaluations of 100 patients entered into an oligonucleotide familial amyloidotic polyneuropathy (FAP) trial. Methods: We assessed: (1) Proficiency of grading neurologic signs and correlation with neurophysiologic tests, and (2) clinometric performance of modified NIS+7 neurophysiologic tests (mNIS+7_Ionis) and its subscores and correlation with disability and health scores. Results: The mNIS+7_Ionis sensitively detected, characterized, and broadly scaled diverse polyneuropathy impairments. Polyneuropathy signs (NIS and subscores) correlated with neurophysiology tests, disability, and health scores. Smart Somatotopic Quantitative Sensation Testing of heat as pain 5 provided a needed measure of small fiber involvement not adequately assessed by other tests. Conclusions: Specially trained neurologists accurately assessed neuropathy signs as compared to referenced neurophysiologic tests. The score, mNIS+7_Ionis, broadly detected, characterized, and scaled polyneuropathy abnormality in FAP, which correlated with disability and health scores. Muscle Nerve 56: 901–911, 2017.

Original language	English (US)
Pages (from-to)	901-911
Number of pages	11
Journal	Muscle and Nerve
Volume	56
Issue number	5
DOIs	https://doi.org/10.1002/mus.25563
State	Published - Nov 2017

Keywords

disability
familial amyloidotic polyneuropathy (FAP)
neurophysiologic tests
oligonucleotide trials
peripheral neuropathy
polyneuropathy signs
proficiency

ASJC Scopus subject areas

Physiology
Clinical Neurology
Cellular and Molecular Neuroscience
Physiology (medical)

Access to Document

10.1002/mus.25563

Cite this

Dyck, P. J., Kincaid, J. C., Dyck, P. J. B., Chaudhry, V., Goyal, N. A., Alves, C., Salhi, H., Wiesman, J. F., Labeyrie, C., Robinson-Papp, J., Cardoso, M., Laura, M., Ruzhansky, K., Cortese, A., Brannagan, T. H., Khoury, J., Khella, S., Waddington-Cruz, M., Ferreira, J., ... Litchy, W. J. (2017). Assessing mNIS+7_Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial. Muscle and Nerve, 56(5), 901-911. https://doi.org/10.1002/mus.25563

Dyck, PJ, Kincaid, JC, Dyck, PJB, Chaudhry, V, Goyal, NA, Alves, C, Salhi, H, Wiesman, JF, Labeyrie, C, Robinson-Papp, J, Cardoso, M, Laura, M, Ruzhansky, K, Cortese, A, Brannagan, TH, Khoury, J, Khella, S, Waddington-Cruz, M, Ferreira, J, Wang, AK, Pinto, MV, Ayache, SS, Benson, MD, Berk, JL, Coelho, T, Polydefkis, M, Gorevic, P, Adams, DH, Plante-Bordeneuve, V, Whelan, C, Merlini, G, Heitner, S, Drachman, BM, Conceição, I, Klein, CJ, Gertz, MA, Ackermann, EJ, Hughes, SG, Mauermann, ML, Bergemann, R, Lodermeier, KA, Davies, JL, Carter, RE & Litchy, WJ 2017, 'Assessing mNIS+7_Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial', Muscle and Nerve, vol. 56, no. 5, pp. 901-911. https://doi.org/10.1002/mus.25563

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title = "Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial",

abstract = "Introduction: Polyneuropathy signs (Neuropathy Impairment Score, NIS), neurophysiologic tests (m+7Ionis), disability, and health scores were assessed in baseline evaluations of 100 patients entered into an oligonucleotide familial amyloidotic polyneuropathy (FAP) trial. Methods: We assessed: (1) Proficiency of grading neurologic signs and correlation with neurophysiologic tests, and (2) clinometric performance of modified NIS+7 neurophysiologic tests (mNIS+7Ionis) and its subscores and correlation with disability and health scores. Results: The mNIS+7Ionis sensitively detected, characterized, and broadly scaled diverse polyneuropathy impairments. Polyneuropathy signs (NIS and subscores) correlated with neurophysiology tests, disability, and health scores. Smart Somatotopic Quantitative Sensation Testing of heat as pain 5 provided a needed measure of small fiber involvement not adequately assessed by other tests. Conclusions: Specially trained neurologists accurately assessed neuropathy signs as compared to referenced neurophysiologic tests. The score, mNIS+7Ionis, broadly detected, characterized, and scaled polyneuropathy abnormality in FAP, which correlated with disability and health scores. Muscle Nerve 56: 901–911, 2017.",

keywords = "disability, familial amyloidotic polyneuropathy (FAP), neurophysiologic tests, oligonucleotide trials, peripheral neuropathy, polyneuropathy signs, proficiency",

author = "Dyck, {Peter J.} and Kincaid, {John C.} and Dyck, {P. James B.} and Vinay Chaudhry and Goyal, {Namita A.} and Christina Alves and Hayet Salhi and Wiesman, {Janice F.} and Celine Labeyrie and Jessica Robinson-Papp and M{\'a}rcio Cardoso and Matilde Laura and Katherine Ruzhansky and Andrea Cortese and Brannagan, {Thomas H.} and Julie Khoury and Sami Khella and M{\'a}rcia Waddington-Cruz and Jo{\~a}o Ferreira and Wang, {Annabel K.} and Pinto, {Marcus V.} and Ayache, {Samar S.} and Benson, {Merrill D.} and Berk, {John L.} and Teresa Coelho and Michael Polydefkis and Peter Gorevic and Adams, {David H.} and Violaine Plante-Bordeneuve and Carol Whelan and Giampaolo Merlini and Stephen Heitner and Drachman, {Brian M.} and Isabel Concei{\c c}{\~a}o and Klein, {Christopher J.} and Gertz, {Morie A.} and Ackermann, {Elizabeth J.} and Hughes, {Steven G.} and Mauermann, {Michelle L.} and Rito Bergemann and Lodermeier, {Karen A.} and Davies, {Jenny L.} and Carter, {Rickey E.} and Litchy, {William J.}",

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year = "2017",

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language = "English (US)",

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T1 - Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial

AU - Dyck, Peter J.

AU - Kincaid, John C.

AU - Dyck, P. James B.

AU - Chaudhry, Vinay

AU - Goyal, Namita A.

AU - Alves, Christina

AU - Salhi, Hayet

AU - Wiesman, Janice F.

AU - Labeyrie, Celine

AU - Robinson-Papp, Jessica

AU - Cardoso, Márcio

AU - Laura, Matilde

AU - Ruzhansky, Katherine

AU - Cortese, Andrea

AU - Brannagan, Thomas H.

AU - Khoury, Julie

AU - Khella, Sami

AU - Waddington-Cruz, Márcia

AU - Ferreira, João

AU - Wang, Annabel K.

AU - Pinto, Marcus V.

AU - Ayache, Samar S.

AU - Benson, Merrill D.

AU - Berk, John L.

AU - Coelho, Teresa

AU - Polydefkis, Michael

AU - Gorevic, Peter

AU - Adams, David H.

AU - Plante-Bordeneuve, Violaine

AU - Whelan, Carol

AU - Merlini, Giampaolo

AU - Heitner, Stephen

AU - Drachman, Brian M.

AU - Conceição, Isabel

AU - Klein, Christopher J.

AU - Gertz, Morie A.

AU - Ackermann, Elizabeth J.

AU - Hughes, Steven G.

AU - Mauermann, Michelle L.

AU - Bergemann, Rito

AU - Lodermeier, Karen A.

AU - Davies, Jenny L.

AU - Carter, Rickey E.

AU - Litchy, William J.

PY - 2017/11

Y1 - 2017/11

N2 - Introduction: Polyneuropathy signs (Neuropathy Impairment Score, NIS), neurophysiologic tests (m+7Ionis), disability, and health scores were assessed in baseline evaluations of 100 patients entered into an oligonucleotide familial amyloidotic polyneuropathy (FAP) trial. Methods: We assessed: (1) Proficiency of grading neurologic signs and correlation with neurophysiologic tests, and (2) clinometric performance of modified NIS+7 neurophysiologic tests (mNIS+7Ionis) and its subscores and correlation with disability and health scores. Results: The mNIS+7Ionis sensitively detected, characterized, and broadly scaled diverse polyneuropathy impairments. Polyneuropathy signs (NIS and subscores) correlated with neurophysiology tests, disability, and health scores. Smart Somatotopic Quantitative Sensation Testing of heat as pain 5 provided a needed measure of small fiber involvement not adequately assessed by other tests. Conclusions: Specially trained neurologists accurately assessed neuropathy signs as compared to referenced neurophysiologic tests. The score, mNIS+7Ionis, broadly detected, characterized, and scaled polyneuropathy abnormality in FAP, which correlated with disability and health scores. Muscle Nerve 56: 901–911, 2017.

AB - Introduction: Polyneuropathy signs (Neuropathy Impairment Score, NIS), neurophysiologic tests (m+7Ionis), disability, and health scores were assessed in baseline evaluations of 100 patients entered into an oligonucleotide familial amyloidotic polyneuropathy (FAP) trial. Methods: We assessed: (1) Proficiency of grading neurologic signs and correlation with neurophysiologic tests, and (2) clinometric performance of modified NIS+7 neurophysiologic tests (mNIS+7Ionis) and its subscores and correlation with disability and health scores. Results: The mNIS+7Ionis sensitively detected, characterized, and broadly scaled diverse polyneuropathy impairments. Polyneuropathy signs (NIS and subscores) correlated with neurophysiology tests, disability, and health scores. Smart Somatotopic Quantitative Sensation Testing of heat as pain 5 provided a needed measure of small fiber involvement not adequately assessed by other tests. Conclusions: Specially trained neurologists accurately assessed neuropathy signs as compared to referenced neurophysiologic tests. The score, mNIS+7Ionis, broadly detected, characterized, and scaled polyneuropathy abnormality in FAP, which correlated with disability and health scores. Muscle Nerve 56: 901–911, 2017.

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KW - oligonucleotide trials

KW - peripheral neuropathy

KW - polyneuropathy signs

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