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杜氏肌营养不良症患儿的失配负波记录:一项整合神经生理学和神经心理学结果的初步研究

Mismatch Negativity Recording in Children With Duchenne Muscular Dystrophy: A Preliminary Study Integrating Neurophysiological and Neuropsychological Results.

作者信息

Filippini Melissa, Guerra Angelo, Negosanti Alessandra, Santi Sara, Sarajlija Jasenka, Musti Muriel Assunta, Gobbi Giuseppe, Lassonde Maryse, Pini Antonella

机构信息

Child Neurology Unit, IRCCS Istituto delle Scienze Neurologiche, Bologna, Italy

Child Neurology Unit, IRCCS Istituto delle Scienze Neurologiche, Bologna, Italy.

出版信息

J Child Neurol. 2016 Nov;31(13):1468-1474. doi: 10.1177/0883073816656404. Epub 2016 Jul 15.

Abstract

Many studies on Duchenne muscular dystrophy children support the hypothesis of a specific neuropsychological phenotype affecting mostly phonological skills. This prospective study aimed to shed light on the role of phonological abilities. Fourteen Duchenne muscular dystrophy children and 7 healthy children underwent mismatch negativity. Moreover, verbal intelligence, visuospatial attention, immediate verbal memory, working memory, grammar, vocabulary, visuomotor skills, reading, text comprehension, writing, and arithmetic were tested in Duchenne muscular dystrophy children. No significant difference between control and Duchenne muscular dystrophy children was found neither for mismatch negativity amplitude (P = .191 and .116, respectively) nor for latency (P = .135). Eight (57.14%) patients showed an impairment of immediate verbal memory and of visuomotor skills, 7 (63.64%) patients had a deficit in writing and arithmetic skills, even with a mean normal intelligence quotient. Taken together, the results put in evidence a heterogeneous neuropsychological profile not explainable on the basis of a phonological deficit.

摘要

许多针对杜兴氏肌营养不良症患儿的研究支持这样一种假说,即存在一种主要影响语音技能的特定神经心理学表型。这项前瞻性研究旨在阐明语音能力的作用。14名杜兴氏肌营养不良症患儿和7名健康儿童接受了失配负波测试。此外,还对杜兴氏肌营养不良症患儿进行了言语智力、视觉空间注意力、即时言语记忆、工作记忆、语法、词汇、视觉运动技能、阅读、文本理解、写作和算术测试。在对照组和杜兴氏肌营养不良症患儿之间,无论是失配负波的波幅(分别为P = 0.191和0.116)还是潜伏期(P = 0.135),均未发现显著差异。8名(57.14%)患者表现出即时言语记忆和视觉运动技能受损;7名(63.64%)患者在写作和算术技能方面存在缺陷,即便其平均智商处于正常水平。综合来看这些结果表明,存在一种无法基于语音缺陷来解释的异质性神经心理学特征。

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