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

Polyneuropathy signs (Neuropathy Impairment Score, NIS), neurophysiologic tests (m+7 ), disability, and health scores were assessed in baseline evaluations of 100 patients entered into an oligonucleotide familial amyloidotic polyneuropathy (FAP) trial. We assessed: (1) Proficiency of grading neurolo...

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Published in:Muscle & nerve 2017-11, Vol.56 (5), p.901-911
Main Authors: 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, 3rd, 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
Format: Article
Language:English
Subjects:
Adult
Aged
Aged, 80 and over
Amyloid Neuropathies, Familial - diagnosis
Amyloid Neuropathies, Familial - drug therapy
Amyloid Neuropathies, Familial - physiopathology
Cohort Studies
Diagnostic Techniques, Neurological
Disability Evaluation
Female
Humans
International Cooperation
Male
Middle Aged
Neural Conduction - drug effects
Neural Conduction - physiology
Neurologists
Oligonucleotides - therapeutic use
Outcome Assessment (Health Care)
Severity of Illness Index
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Summary:Polyneuropathy signs (Neuropathy Impairment Score, NIS), neurophysiologic tests (m+7 ), disability, and health scores were assessed in baseline evaluations of 100 patients entered into an oligonucleotide familial amyloidotic polyneuropathy (FAP) trial. 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 ) and its subscores and correlation with disability and health scores. The mNIS+7 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. Specially trained neurologists accurately assessed neuropathy signs as compared to referenced neurophysiologic tests. The score, mNIS+7 , broadly detected, characterized, and scaled polyneuropathy abnormality in FAP, which correlated with disability and health scores. Muscle Nerve 56: 901-911, 2017.
ISSN:0148-639X
1097-4598
DOI:10.1002/mus.25563