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贝克型肌营养不良症和良性婴儿型脊髓性肌萎缩症的骨骼肌计算机断层扫描

Computed tomography of the skeletal musculature in Becker-type muscular dystrophy and benign infantile spinal muscular atrophy.

作者信息

de Visser M, Verbeeten B

机构信息

Muscle Research Centre, Department of Neurology, University of Amsterdam, The Netherlands.

出版信息

Muscle Nerve. 1985 Jun;8(5):435-44. doi: 10.1002/mus.880080514.

DOI:10.1002/mus.880080514
PMID:16758591
Abstract

Results of computed tomographic (CT) examination of the skeletal musculature in 26 patients with Becker-type muscular dystrophy (BMD) and 12 patients with benign infantile spinal muscular atrophy (BISMA) are presented. Both disorders revealed strikingly different changes that may have important clinical significance. First, in BMD, CT abnormalities consisted of areas of decreased densities, at first appearing in part of the muscle, and then gradually spreading until the whole muscle was replaced by low-density tissue. In BISMA, however, low-density lesions were scattered throughout the muscle. Second, in BMD, some muscles were preferentially affected in an early stage of the disease and others were relatively spared, whereas in BISMA, the muscles were involved more or less simultaneously. Third, an enlargement in size (hypertrophy) was frequently observed in BMD in various muscles of the legs, whereas in BISMA, this phenomenon can sometimes be noted only in the gastrocnemius muscles.

摘要

本文展示了对26例贝克型肌营养不良症(BMD)患者和12例良性婴儿型脊髓性肌萎缩症(BISMA)患者骨骼肌的计算机断层扫描(CT)检查结果。这两种疾病显示出截然不同的变化,可能具有重要的临床意义。首先,在BMD中,CT异常表现为密度降低区域,最初出现在部分肌肉中,然后逐渐扩散,直到整个肌肉被低密度组织取代。然而,在BISMA中,低密度病变散在分布于整个肌肉。其次,在BMD中,某些肌肉在疾病早期优先受累,而其他肌肉相对幸免,而在BISMA中,肌肉或多或少同时受累。第三,在BMD中,经常观察到腿部各肌肉尺寸增大(肥大),而在BISMA中,这种现象有时仅在腓肠肌中可见。

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"Quadriceps myopathy": a clinical variant form of Becker muscular dystrophy.
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