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Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial.
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, 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.
Afiliação
  • Dyck PJ; Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
  • Kincaid JC; Indiana University, IU Health, Indianapolis, Indiana, USA.
  • Dyck PJB; Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
  • Chaudhry V; Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
  • Goyal NA; University of California Irvine, Irvine, California, USA.
  • Alves C; Hospital Santo António, Centro Hospitalar Porto, Porto, Portugal.
  • Salhi H; Hôpitaux Universitaires Henri Mondor, Créteil, France.
  • Wiesman JF; Boston Medical Center, Boston, Massachusetts, USA.
  • Labeyrie C; CHU Bicêtre, French Reference Center for FAP (NNERF), Le Kremlin Bicêtre, France.
  • Robinson-Papp J; Icahn School of Medicine at Mount Sinai, New York, New York, USA.
  • Cardoso M; Hospital Santo António, Centro Hospitalar Porto, Porto, Portugal.
  • Laura M; National Hospital for Neurology and Neurosurgery, London, United Kingdom.
  • Ruzhansky K; Medical University of South Carolina, Charleston, South Carolina, USA.
  • Cortese A; C. Mondino National Neurological Institute, Pavia, Italy.
  • Brannagan TH; Columbia University, New York, New York, USA.
  • Khoury J; Oregon Health & Science University, Portland, Oregon, USA.
  • Khella S; Penn Presbyterian Medical Center, Philadelphia, Pennsylvania, USA.
  • Waddington-Cruz M; Clementino Fraga Filho University Hospital, Federal University of Rio de Janerio, Rio de Janerio, Brazil.
  • Ferreira J; New University of Lisbon, Lisbon, Portugal.
  • Wang AK; University of California-Irvine, Orange, California, USA.
  • Pinto MV; Clementino Fraga Filho University Hospital, Federal University of Rio de Janerio, Rio de Janerio, Brazil.
  • Ayache SS; Hôpitaux Universitaires Henri Mondor, Créteil, France.
  • Benson MD; Indiana University School of Medicine, Indianapolis, Indiana, USA.
  • Berk JL; Boston University School of Medicine, Boston, Massachusetts, USA.
  • Coelho T; Hospital Santo António, Centro Hospitalar Porto, Porto, Portugal.
  • Polydefkis M; Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
  • Gorevic P; Icahn School of Medicine at Mount Sinai, New York, New York, USA.
  • Adams DH; CHU Bicêtre, French Reference Center for FAP (NNERF), Le Kremlin Bicêtre, France.
  • Plante-Bordeneuve V; Hôpitaux Universitaires Henri Mondor, Créteil, France.
  • Whelan C; National Hospital for Neurology and Neurosurgery, London, United Kingdom.
  • Merlini G; Foundation IRCCS Policlinico San Matteo, University of Pavia, Italy.
  • Heitner S; Oregon Health & Science University, Portland, Oregon, USA.
  • Drachman BM; Penn Presbyterian Medical Center, Philadelphia, Pennsylvania, USA.
  • Conceição I; New University of Lisbon, Lisbon, Portugal.
  • Klein CJ; Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
  • Gertz MA; Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
  • Ackermann EJ; Ionis Pharmaceuticals, Inc, Carlsbad, California, USA.
  • Hughes SG; Ionis Pharmaceuticals, Inc, Carlsbad, California, USA.
  • Mauermann ML; Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
  • Bergemann R; GlaxoSmithKline, Middlesex, United Kingdom.
  • Lodermeier KA; Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
  • Davies JL; Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
  • Carter RE; Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
  • Litchy WJ; Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
Muscle Nerve ; 56(5): 901-911, 2017 Nov.
Article em En | MEDLINE | ID: mdl-28063170
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.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Oligonucleotídeos / Índice de Gravidade de Doença / Neuropatias Amiloides Familiares / Técnicas de Diagnóstico Neurológico / Neurologistas Idioma: En Ano de publicação: 2017 Tipo de documento: Article
Texto completo
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Oligonucleotídeos / Índice de Gravidade de Doença / Neuropatias Amiloides Familiares / Técnicas de Diagnóstico Neurológico / Neurologistas Idioma: En Ano de publicação: 2017 Tipo de documento: Article