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

Dyck, PJ; Kincaid, JC; Dyck, PJB; Chaudhry, V; Goyal, NA; Alves, C; Salhi, H; ... Litchy, WJ; + view all (2017) Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial. Muscle Nerve , 56 (5) pp. 901-911. 10.1002/mus.25563. Green open access

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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. Muscle Nerve 56: 901-911, 2017.

Type: Article
Title: Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1002/mus.25563
Publisher version: https://doi.org/10.1002/mus.25563
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Disability, familial amyloidotic polyneuropathy (FAP), neurophysiologic tests, oligonucleotide trials, peripheral neuropathy, polyneuropathy signs, proficiency, Adult, Aged, Aged, 80 and over, Amyloid Neuropathies, Familial, Cohort Studies, Diagnostic Techniques, Neurological, Disability Evaluation, Female, Humans, International Cooperation, Male, Middle Aged, Neural Conduction, Neurologists, Oligonucleotides, Outcome Assessment (Health Care), Severity of Illness Index
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/10077908
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