偏侧萎缩症的临床与病理研究(附1例报道及文献复习)

Clinical and pathological feature in a patient with hemiatrophy

目的了解偏侧萎缩症的临床特点、病理组织学和超微结构改变。方法对1例单纯右下肢萎缩患者的临床表现、实验室检查、组织病理学及超微结构进行分析。结果患者右下肢皮肤变薄,皮下组织几乎消失,肌肉轻度萎缩,骨骼变短,神经系统检查和相关实验室检查均无异常,患肢皮肤和肌肉活检在光镜下见肌纤维散在萎缩,肌膜炎性细胞浸润,血管壁明显增厚,管腔狭窄,皮肤各层组织变薄,皮下脂肪组织减少;电镜下见在有病变的肌纤维内线粒体减少并空泡样变,肌细胞胞核数目减少,胞核周围胞浆内可见异常颗粒沉积。结论单纯一个肢体萎缩可能是偏侧萎缩症的一个特殊类型。

Objective To illustrate the clinical features, pathologic histology and the change of ultramicrostructure of hemiatrophy. Methods We analyzed the clinical manifestations, laboratory examinations, pathologic histology and ultramicrostructure in a case of hemiatrophy who had pure right lower limb atrophy. Resuits In the right lower limb we found the thinner skin, the nearly disappeared hypoderma, the mild muscle atrophy and short bones. There was no abnormities in neurologic examination and laboratory findings. Under the light microscope, the skin and muscle of the affected limbs showed the scattered atrophy of muscle fiber, the infiltration of inflammatory cells in tunica muscularis, the obvious thickening of vessel wall and the narrow lumina. We also observed the thinning of every layer of skin and the reduction of subcutaneous fatty tissue. Electron microscope showed the decreased mitochondria in the pathological muscle fibers. Furthermore, wo also found the vacuolus degeneration in the same fiber. In the same muscle cells, the nuclei were rare and abnormal granules deposited in cytoplasm around nuclei. Conclusions A single limb atrophy may be a special type of hemiatrophy.