目的：分析肌萎缩性双侧臂瘫的临床特征及病因，提高对该病的认识。方法通过询问病史、体格检查、神经电生理学检查，并结合文献复习，对该病的临床特征和病因进行分析。结果肌萎缩性双侧臂瘫患者表现为双上肢近端显著无力和双侧上肢带肌明显萎缩，而双下肢功能较少受损。该病进展较经典的肌萎缩侧索硬化相对缓慢、女性罕见，发病原因可能与基因突变相关。结论肌萎缩性双侧臂瘫的临床特征和病因学分析对其筛查和早期诊断具有重要价值。%Objective To analyze the clinical features and etiology of brachial amyotrophic diplegia and raise the awareness of this disease.Methods Analyze the clinical features and etiology of the disease by carefully asking the history, making physical examination and clinical electric neurophysiology examination and combining with the literature review. Results The patients with brachial amyotrophic diplegia have the features that the ends of their both upper limbs are signiifcantly weak and the muscles of their bilateral upper limbs are signiifcantly atrophied, with little or no functional impairment of the legs. However, compared with classical upper limb onset amyotrophic lateral sclerosis, this disease makes slower progress and is rarely found on women. The etiology may be related to gene mutation.Conclusion The analysis of the clinical features and etiology of brachial amyotrophic diplegia has a signiifcant value in its screening and early clinical diagnosis.
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