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杜兴氏和贝克氏型肌营养不良症的比较分析

Comparative analysis between Duchenne and Becker types muscular dystrophy.

作者信息

Ishpekova B, Milanov I, Christova L G, Alexandrov A S

机构信息

Department of Neurology, University Hospital Tzarita Ioanna, Sofia, Bulgaria.

出版信息

Electromyogr Clin Neurophysiol. 1999 Jul-Aug;39(5):315-8.

Abstract

Duchenne and Becker types of muscular dystrophy are usually differentiated according to age of onset and rate of progression criteria which are not sufficient. The aim of this paper was to re-establish the clues for distinguishing Duchenne from Becker types of muscular dystrophy. According to the onset and progression of the disease, one hundred and eleven patients were subdivided into two groups. First group--Becker muscular dystrophy--consisted of 40 patients and second one of 71 patients with Duchenne type of muscular dystrophy. Clinical data confirm some well known differences between Duchenne and Becker muscular dystrophy concerning the age of onset, severity of disease and rate of progression. Electromyographic signs of myopathic changes and spontaneous activity were found in both diseases. Spontaneous activity--bizarre and fibrillation potentials, as well as sharp waves are more common for Duchenne type. The differences between the Becker from Duchenne type of muscular dystrophy can be described on the basis of complex investigations (clinical, electromyographical, histological and biochemical).

摘要

杜兴氏和贝克氏型肌营养不良症通常根据发病年龄和进展速度标准进行区分,而这些标准并不充分。本文的目的是重新确立区分杜兴氏和贝克氏型肌营养不良症的线索。根据疾病的发病和进展情况,111例患者被分为两组。第一组——贝克氏肌营养不良症——由40例患者组成,第二组由71例杜兴氏型肌营养不良症患者组成。临床数据证实了杜兴氏和贝克氏肌营养不良症在发病年龄、疾病严重程度和进展速度方面一些众所周知的差异。两种疾病均发现了肌病性改变和自发活动的肌电图征象。自发活动——奇异电位和纤颤电位以及锐波在杜兴氏型中更为常见。贝克氏型和杜兴氏型肌营养不良症之间的差异可以通过综合检查(临床、肌电图、组织学和生化)来描述。

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