以往曾报道,在肌萎缩侧索硬化患者中,生理刺激引起的心血管反应是正常的。本文作者则报道1例肌萎缩侧索硬化患者其植物神经心血管反应正常,但有交感胆碱能神经功能障碍。患者为63岁男性,进行性力弱2.5年。首先出现右下肢力弱,逐渐累及右上肢,然后累及左侧肢体。入院前一个月出现言语不清。无吞咽困难以及皮肤出汗量及分布异常。神经系统检查可见舌及四肢肌肉有束颤。全身肌力减弱,以右侧为著。全身肌肉有轻至中度萎缩,尤以手固有肌明显,亦以右侧为 It has been previously reported that in patients with amyotrophic lateral sclerosis, cardiovascular responses to physiological stimuli are normal. The authors report that one patient with amyotrophic lateral sclerosis had normal autonomic cardiovascular response, but had sympathetic cholinergic dysfunction. The patient was 63 years old and had a weak sexual performance of 2.5 years. First, the right lower limb weakness, gradually involving the right upper limb, and then involving the left limb. One month before admission, his speech was unclear. No difficulty swallowing and skin sweat volume and distribution abnormalities. Nerve system examination shows tongue and limb muscle bundle vibration. Body weakness, with the right side. Body muscle mild to moderate atrophy, especially hand obvious muscle, also to the right