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Muscle Nerve. 2017 Nov;56(5):901-911. doi: 10.1002/mus.25563. Epub 2017 Apr 7.

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

Author information

1
Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
2
Indiana University, IU Health, Indianapolis, Indiana, USA.
3
Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
4
University of California Irvine, Irvine, California, USA.
5
Hospital Santo António, Centro Hospitalar Porto, Porto, Portugal.
6
Hôpitaux Universitaires Henri Mondor, Créteil, France.
7
Boston Medical Center, Boston, Massachusetts, USA.
8
CHU Bicêtre, French Reference Center for FAP (NNERF), Le Kremlin Bicêtre, France.
9
Icahn School of Medicine at Mount Sinai, New York, New York, USA.
10
National Hospital for Neurology and Neurosurgery, London, United Kingdom.
11
Medical University of South Carolina, Charleston, South Carolina, USA.
12
C. Mondino National Neurological Institute, Pavia, Italy.
13
Columbia University, New York, New York, USA.
14
Oregon Health & Science University, Portland, Oregon, USA.
15
Penn Presbyterian Medical Center, Philadelphia, Pennsylvania, USA.
16
Clementino Fraga Filho University Hospital, Federal University of Rio de Janerio, Rio de Janerio, Brazil.
17
New University of Lisbon, Lisbon, Portugal.
18
University of California-Irvine, Orange, California, USA.
19
Indiana University School of Medicine, Indianapolis, Indiana, USA.
20
Boston University School of Medicine, Boston, Massachusetts, USA.
21
Foundation IRCCS Policlinico San Matteo, University of Pavia, Italy.
22
Ionis Pharmaceuticals, Inc, Carlsbad, California, USA.
23
GlaxoSmithKline, Middlesex, United Kingdom.

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.

KEYWORDS:

disability; familial amyloidotic polyneuropathy (FAP); neurophysiologic tests; oligonucleotide trials; peripheral neuropathy; polyneuropathy signs; proficiency

PMID:
28063170
PMCID:
PMC5500439
DOI:
10.1002/mus.25563
[Indexed for MEDLINE]
Free PMC Article

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