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遗传性脊髓近端肌萎缩症(Hereditary proximal spinal muscular atrophy)是一种少见的神经肌肉系统遗传性疾病。发病年龄多在1～17岁,初表现为下肢和躯干部的肌肉萎缩,渐发展至全身。近端肌肉萎缩较远端为重。病人感觉正常,病程可长达10～40年,最
Hereditary proximal spinal muscular atrophy is a rare genetic disease of the neuromuscular system. Age more than 1 to 17 years of age, initially manifested as muscular atrophy of the lower extremities and trunk, gradually developing to the body. Near the distal muscle atrophy more serious. The patient feels normal, duration of up to 10 to 40 years, most