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 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

    Research output: Contribution to journalArticle

    17 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

    Dyck, P. J., Kincaid, J. C., Dyck, P. J. B., Chaudhry, V., Goyal, N. A., Alves, C., ... Litchy, W. J. (Accepted/In press). Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial. Muscle and Nerve. https://doi.org/10.1002/mus.25563

    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 J.; Gertz, Morie A.; Ackermann, Elizabeth J.; Hughes, Steven G.; Mauermann, Michelle L.; 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, MA, Ackermann, EJ, Hughes, SG, Mauermann, ML, 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 J. ; Gertz, Morie A. ; Ackermann, Elizabeth J. ; Hughes, Steven G. ; Mauermann, Michelle L. ; 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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    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.",
    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{\cc}{\~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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    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

    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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