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Assessing mNIS+7 Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial
Author(s) -
Dyck Peter J.,
Kincaid John C.,
Dyck P. James B.,
Chaudhry Vinay,
Goyal Namita A.,
Alves Christina,
Salhi Hayet,
Wiesman Janice F.,
Labeyrie Celine,
RobinsonPapp Jessica,
Cardoso Márcio,
Laura Matilde,
Ruzhansky Katherine,
Cortese Andrea,
Brannagan Thomas H.,
Khoury Julie,
Khella Sami,
WaddingtonCruz 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.,
PlanteBordeneuve 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.
Publication year - 2017
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.25563
Subject(s) - polyneuropathy , medicine , physical therapy , abnormality , physical medicine and rehabilitation , psychiatry
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