僵人综合征4例分析及文献回顾

Clinical characteriscs of stiff-person syndrome

目的总结僵人综合征的临床特点,提高对该病的认识和诊断水平。方法分析4例病例,总结其临床表现、典型的肌电图表现及治疗效果。结果 4例患者3名男性,1名女性,年龄29岁~51岁,1例为受凉约2w后出现症状,3例无明显诱发因素。4例既往均无特殊疾病史,亦无家族同类病史。2例为亚急性起病,2例为慢性起病,病程分别为1~13个月。主要表现为躯干、四肢近端肌肉出现阵发或持续性僵硬和疼痛,从而引起相应部位的运动障碍。4例均行常规肌电图检查,显示在放松状态下时有持续运动单位电位,应用安定后消失。经苯二氮草类、巴氯芬治疗后2例可痊愈,另2例亦无反复发作及恶化。结论僵人综合征是一种罕见的肌肉疾病,易误诊,苯二氮草类、巴氯芬治疗可获良好效果。

Objective The clinical features of stiff-person syndrome（SPS） were described in order to improve the diagnosis.Methods The clinical manifestations,typical electromyogram（EMG） findings and the therapeutic effects of the 4 cases were summarize.Results In the 4 cases,3 were males and 1 was female.Their ages were between 29 to 51.One case had the onset of the disease after catching a cold,other cases had no inducement.No special past history or family history was found.2 cases had subacute onset and 2 had chronic onset of the disease.The durations of the disease were between 1 to 13 months.The chief clinical manifestations were paroxysmal or persistant stiffness and pain in the muscles of proximal limbs and trunk,and then dyskinesia of the relevant sites.Persistant MUAPs were recorded in EMG at rest,but disappeared after valium injection.After treated by benzodiazepines and baclofen,2 cases were recovered,and 2 cases had no progression of the symptoms.Conclusions Stiff-person syndrome is a rare muscle disease which is easy to mistake in diagnosis.Benzodiazepines and baclofen could have good curative effect.