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肌萎缩侧索硬化(amyotrophic laterral sclerosis, ALS)是一种病因尚未明确,选择性侵犯脊髓前角细胞、脑干运动神经元、皮质锥体细胞及锥体束的致命的慢性进行性变性疾病,其病理特征为进行性上、下运动神经元的变性,临床以上或/和下运动神经元损害引起的肌无力、肌肉萎缩和锥体束征的不同组合为主要表现,患者最终因呼吸衰竭致死,感觉和括约肌功能一般不受影响。ALS目前尚无特异性诊断指标,电生理、影像方面可提供帮助。
Amyotrophic lateral sclerosis (ALS) is a deadly chronic progressive degenerative disease of unknown etiology that selectively affects the anterior horn cells of the spinal cord, brainstem motor neurons, cortical pyramidal cells, and the pyramidal tract, The pathological features of progressive upper and lower motor neuron degeneration, clinical and / or lower motor neuron damage caused by muscle weakness, muscle atrophy and pyramidal tract signs of the different combinations as the main performance, the patient eventually died of respiratory failure , Sensory and sphincter functions are generally unaffected. ALS currently no specific diagnostic indicators, electrophysiology, imaging can provide help.