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 journalArticlepeer-review

29 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)901-911
Number of pages11
JournalMuscle and Nerve
Issue number5
StatePublished - Nov 2017


  • 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)


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