僵人综合征——附5例报告并文献复习

Stiff-man syndrome: clinical analysis of 5 cases

目的探讨僵人综合征的临床特点,提高对该病的认识。方法分析5例病例,并复习文献。结果本组病例均为男性,年龄13～55岁,急性或亚急性起病,主要症状为躯干及四肢肌肉僵硬伴肌痉挛,腹肌、咀嚼肌、表情肌和呼吸肌受累也不少见,伴有或不伴有疼痛。肌电图显示肌休时有持续运动单位电位,应用安定后消失。本组住院17～105天,恢复完全或不完全。结论我国僵人综合征临床表现有自身特点,青壮年男性多见,起病较急,进展较快。痉笑面容、牙关紧闭对僵人综合征与破伤风无鉴别诊断意义。安定治疗效果满意。

ObjectiveTo investigate the clinical features of Chinese patients with stiff-man syndrome (SMS) and improve theunderstanding to the rare disease. Methods Five cases of male SMS from 1977 to 1998 were analyzed retrospectively. Therelevant literature was reviewed.ResultsSymptoms began between the age of 13 and 55 (average 38) with an acute or sub-acuteonset. The course varied from weeks to months. Muscle stiffness of spine and extremities with superimposed spasms were the primarysymptoms. Masseter, facial and respiratory muscles were also frequently involved. Electromyography showed continuous motor activ-ity which was abolished by diazepam. Conclusions SMS in Chinese has itself peculiarity in symptoms. Sardonic grin and trismushas no value in the distinguishment of SMS and tetanus. EMG is an important diagnostic tool and benzodiazepine is as yet the mosteffective therapy.