在一项家族性淀粉样多发性神经病试验中评估改良神经病变损害评分+7（mNIS+7）及国际神经科医生的专业水平。
Assessing mNIS+7 and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial.
作者信息
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
机构信息
Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
Indiana University, IU Health, Indianapolis, Indiana, USA.
出版信息
Muscle Nerve. 2017 Nov;56(5):901-911. doi: 10.1002/mus.25563. Epub 2017 Apr 7.
INTRODUCTION
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.
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 ) and its subscores and correlation with disability and health scores.
RESULTS
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.
CONCLUSIONS
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.
引言
在一项针对100名参与寡核苷酸家族性淀粉样多神经病（FAP）试验的患者进行的基线评估中，对多神经病体征（神经病变损伤评分，NIS）、神经生理学测试（m + 7）、残疾情况和健康评分进行了评估。
方法
我们评估了：（1）神经体征分级的熟练度及其与神经生理学测试的相关性，以及（2）改良的NIS + 7神经生理学测试（mNIS + 7）及其子评分的临床计量学表现及其与残疾和健康评分的相关性。
结果
mNIS + 7能够灵敏地检测、表征并广泛衡量各种多神经病损伤。多神经病体征（NIS及其子评分）与神经生理学测试、残疾情况和健康评分相关。将热作为疼痛的智能躯体感觉定量测试5提供了一种其他测试未充分评估的小纤维受累情况的必要测量方法。
结论
与参考神经生理学测试相比，经过专门培训的神经科医生能够准确评估神经病体征。mNIS + 7评分能够广泛检测、表征并衡量FAP中的多神经病异常情况，且与残疾和健康评分相关。《肌肉与神经》2017年第56卷：901 - 911页。
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