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. 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 Jon Klein, Morie Gertz, Elizabeth J. Ackermann, Steven G. Hughes, Michelle M Mauermann, Rito Bergemann, Karen A. Lodermeier, Jenny L. Davies, Rickey E. Carter, William J Litchy

Research output: Contribution to journalArticle

19 Citations (Scopus)

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.

Original languageEnglish (US)
JournalMuscle and Nerve
DOIs
StateAccepted/In press - 2017

Fingerprint

Polyneuropathies
Health
Neurophysiology
Neurologic Manifestations
Oligonucleotides
Neurologists
Hot Temperature
Pain

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)

Cite this

Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial. / Dyck, Peter J; Kincaid, John C.; Dyck, P. James B; Chaudhry, Vinay; Goyal, Namita A.; Alves, Christina; Salhi, Hayet; Wiesman, Janice F.; Labeyrie, Celine; Robinson-Papp, Jessica; Cardoso, Márcio; Laura, Matilde; Ruzhansky, Katherine; Cortese, Andrea; Brannagan, Thomas H.; Khoury, Julie; Khella, Sami; Waddington-cruz, Márcia; Ferreira, João; Wang, Annabel K.; Pinto, Marcus V.; Ayache, Samar S.; Benson, Merrill D.; Berk, John L.; Coelho, Teresa; Polydefkis, Michael; Gorevic, Peter; Adams, David H.; Plante-Bordeneuve, Violaine; Whelan, Carol; Merlini, Giampaolo; Heitner, Stephen; Drachman, Brian M.; Conceição, Isabel; Klein, Christopher Jon; Gertz, Morie; Ackermann, Elizabeth J.; Hughes, Steven G.; Mauermann, Michelle M; Bergemann, Rito; Lodermeier, Karen A.; Davies, Jenny L.; Carter, Rickey E.; Litchy, William J.

In: Muscle and Nerve, 2017.

Research output: Contribution to journalArticle

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, M, Ackermann, EJ, Hughes, SG, Mauermann, MM, Bergemann, R, Lodermeier, KA, Davies, JL, Carter, RE & Litchy, WJ 2017, 'Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial', Muscle and Nerve. https://doi.org/10.1002/mus.25563
Dyck, Peter J ; Kincaid, John C. ; Dyck, P. James B ; Chaudhry, Vinay ; Goyal, Namita A. ; Alves, Christina ; Salhi, Hayet ; Wiesman, Janice F. ; Labeyrie, Celine ; Robinson-Papp, Jessica ; Cardoso, Márcio ; Laura, Matilde ; Ruzhansky, Katherine ; Cortese, Andrea ; Brannagan, Thomas H. ; Khoury, Julie ; Khella, Sami ; Waddington-cruz, Márcia ; Ferreira, João ; Wang, Annabel K. ; Pinto, Marcus V. ; Ayache, Samar S. ; Benson, Merrill D. ; Berk, John L. ; Coelho, Teresa ; Polydefkis, Michael ; Gorevic, Peter ; Adams, David H. ; Plante-Bordeneuve, Violaine ; Whelan, Carol ; Merlini, Giampaolo ; Heitner, Stephen ; Drachman, Brian M. ; Conceição, Isabel ; Klein, Christopher Jon ; Gertz, Morie ; Ackermann, Elizabeth J. ; Hughes, Steven G. ; Mauermann, Michelle M ; Bergemann, Rito ; Lodermeier, Karen A. ; Davies, Jenny L. ; Carter, Rickey E. ; Litchy, William J. / Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial. In: Muscle and Nerve. 2017.
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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.",
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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 Jon

AU - Gertz, Morie

AU - Ackermann, Elizabeth J.

AU - Hughes, Steven G.

AU - Mauermann, Michelle M

AU - Bergemann, Rito

AU - Lodermeier, Karen A.

AU - Davies, Jenny L.

AU - Carter, Rickey E.

AU - Litchy, William J

PY - 2017

Y1 - 2017

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.

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.

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KW - Familial amyloidotic polyneuropathy (FAP)

KW - Neurophysiologic tests

KW - Oligonucleotide trials

KW - Peripheral neuropathy

KW - Polyneuropathy signs

KW - Proficiency

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