Objective To investigate the differences of the pyramidal sign on amyotrophic lateral sclerosis ( ALS ) and cervical spondilotic myelopathy ( CSM ). Methods There were 50 cases of ALS and 50 cases of CSM enrolled. The differences of the appearance rates of the original symptoms and the clinical signs as determined by visiting doctor, such as the pain in the neck and shoulders, the atrophy with fasciculations and fibrillations of tongue muscle and pathological signs were compared between the two groups. Results The appearance rates of the weakness of lower extremities was the first symptoms of ALS, the ball paralysis and the atrophy with fasciculations and fibrillations of tongue muscle and the disappearance of tendon reflex of ALS as visiting doctor were higher than those of CSM ( P <0. 01 ). Whereas the appearance rates of the weakness of upper extremities was the first symptoms of CSM with the pain in the neck and shoulders and the pathological signs as visiting doctor, such as Babinski sign, Shodock sign and Openham sign of CSM, were higher than those of ALS ( P <0. 01 ). In both group of cases, there were weakness and atrophy of the extremities. And the clinical signs were elicited in all cases such as the tendon hyperreflexia, the clonus of patellar or ankle and the signs of Rossolimo and Hoffmann. Conclusion There may be appeared deep hyperreflexia in patient with either ALS or CSM, but the positive rate of pathological sign is much lower than that of the patient with CSM,with the lowest positive rate of Babinski sign.%目的 探讨肌萎缩性侧索硬化症(ALS)和脊髓型颈椎病(CSM)锥体束征的差异.方法 纳入ALS和CSM患者各50例,详细询问病史、神经系统检查,行头颈部MR、四肢和胸锁乳突肌肌电图检查;分别比较2组患者的首发症状,就诊时颈肩疼痛、舌肌萎缩、肌束颤动及病理征等临床征象阳性率的差异.结果 ALS组的首发症状以下肢无力和延髓麻痹为主,就诊时舌肌委缩、肌束颤动、跖反射消失出现率较高;而CSM组的首发症状以上肢无力为主,就诊时颈肩疼痛和巴宾斯基征、夏道克征、欧本海姆征等病理征的阳性率较高(P<0.01).2组病例均有四肢肌无力、萎缩(CSM组下肢肌萎缩较少)、腱反射亢进、髌和踝阵挛、罗索里摩征及霍夫曼征(+).结论 ALS和CSM患者均可出现深反射亢进,但ALS患者的病理征阳性率远低于CSM,以巴宾斯基征的阳性率最低.
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