| 脂质沉积性肌病的临床及病理学特点 |
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| 引用本文： | 沈宏锐,胡静,赵哲,李娜,袁军辉,邴琪,张江涛.脂质沉积性肌病的临床及病理学特点[J].临床神经病学杂志,2009,22(3). |
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| 作者姓名： | 沈宏锐 胡静 赵哲 李娜 袁军辉 邴琪 张江涛 |
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| 作者单位： | 河北医科大学第三医院神经肌肉病科,石家庄,050051 |
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| 摘 要： | 目的探讨脂质沉积性肌病(LSM)的临床及病理学特点。方法对14例LSM患者的临床及骨骼肌病理资料进行分析。结果14例LSM患者的主要临床表现为以近端肌无力起病,渐累及全身,其中13例四肢受累,1例双下肢受累,2例颈肌受累,伴肌痛2例、肌萎缩3例;血清肌酸激酶正常5例,不同程度增高9例;14例肌电图均呈肌源性损害;14例骨骼肌病理检查(HE、MGT染色)可见大量肌纤维胞浆内散在空泡,油红"O"染色空泡红染;透射电镜分析可见沿肌纤维长轴散在串珠样膜性空泡结构。结论LSM临床表现为非特异性肌无力;肌纤维内脂质沉积是LSM主要病理表现;骨骼肌病理检查是确诊LSM的可靠方法。
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| 关 键 词： | 脂质沉积性肌病 骨骼肌活检 病理学 透射电镜 |
Clinical and pathological features of lipid storage myopathy |
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| SHEN Hong-rui,HU Jing,ZHAO Zhe,et al..Clinical and pathological features of lipid storage myopathy[J].Journal of Clinical Neurology,2009,22(3). |
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| Authors: | SHEN Hong-rui HU Jing ZHAO Zhe |
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| Institution: | SHEN Hong-rui,HU Jing,ZHAO Zhe,et al.Department of Neuromuscular,the Third Hospital,Hebei Medical University,Shijiazhuang 050051,China |
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| Abstract: | Objective To study the clinical and pathological features of lipid storage myopathy(LSM).Methods The clinical and skeletal muscle pathologital data of 14 patients with LSM were analysed.Results Main clinical manifestations of the 14 LSM patients were progressive muscle weakness from proximal to general,including 13 cases involved extremities,1 case involoved double lower limbs,2 cases involved muscles of neck.It could accompanied with muscle pain(2 cases) and muscle atrophy(3 cases).The levels of serum crea... |
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| Keywords: | lipid storage myopathy skeletal muscle biopsy pathology transmission electromicroscope |
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