Comparative analysis between Duchenne and Becker types muscular dystrophy.

Comparative analysis between Duchenne and Becker types muscular dystrophy.
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Duchenne 型和 Becker 型肌营养不良症的比较分析。

DOI:
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发表时间:
1999
期刊:
Electromyography and clinical neurophysiology
影响因子:
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通讯作者:
A. S. Alexandrov
A. S. Alexandrov
中科院分区:
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文献类型:
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作者:
B. Ishpekova;I. Milanov;Christova Lg;A. S. Alexandrov

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杜氏型和贝克尔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,但这些标准并不充分。本文的目的是重新建立区分 Duchenne 型和 Becker 型肌营养不良症的线索。根据疾病的发作和进展,111名患者被分为两组。第一组——贝克尔肌营养不良症——由 40 名患者组成,第二组由 71 名杜兴型肌营养不良症患者组成。临床数据证实了杜氏肌营养不良症和贝克尔肌营养不良症在发病年龄、疾病严重程度和进展速度方面的一些众所周知的差异。在这两种疾病中都发现了肌病变化和自发活动的肌电图征象。自发活动——奇怪的颤动电位以及尖锐的波在杜氏型中更为常见。 Becker 型和 Duchenne 型肌营养不良症之间的差异可以在复杂的研究(临床、肌电图、组织学和生化)的基础上进行描述。
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).