进行性脊肌萎缩症 [1]是神经系统疾病中运动神经细胞变性坏死的疾病 ,属于运动神经元病的一种。运动神经元变性只限于脊髓前角细胞而不累及上运动神经元时 ,称为进行性脊肌萎缩症。本病较少见 ,萎缩可能局限于一部分肌肉 ,历时数月甚至一二年 ,或者可以缓慢或迅速地波及全身 ,? Progressive spinal muscular atrophy [1] is a neurodegenerative disease of motor nerve cells degeneration and necrosis, is a motor neuron disease. Motor neuron degeneration is limited to spinal cord anterior horn cells without involvement of upper motor neurons, known as progressive spinal muscular atrophy. The disease is rare, atrophy may be limited to part of the muscle, which lasted several months or even a year or two, or can spread slowly or rapidly throughout the body?