患者许× ×，男，７４岁，退休干部。主诉：进行性双上肢肌肉萎缩，活动不利３年多。患者于３年前无明显诱因出现右上肢麻木及拇指僵硬感，不伴疼痛。３月后，出现双上肢远端肌肉萎缩，尤以大、小鱼际、骨间肌萎缩明显，双手握拳困难，活动不利。当时未予以重视，１９９８年７ Xu × × patients, male, 74 years old, retired cadres. Chief Complaint: progressive upper extremity muscular atrophy, adverse events more than 3 years. Patients with no obvious incentive to appear 3 years ago numbness of the upper right leg and stiff sense of the thumb, without pain. 3 months later, there are distal upper extremity muscle atrophy, especially in large, small intertial, interscaleal muscle atrophy was obvious, both hands fist difficulties, adverse events. At that time did not pay attention, in 1998 7