僵肢综合征临床分析

目的 探讨僵肢综合征（SLS）临床及电生理特点。方法 对1例僵肢综合征临床资料进行分析。并综合文献。阐述该病的临床及电生理特点。治疗方案及预后。结果 僵肢综合征是僵入综合征的变异型的一种，多与自身免疫及副肿瘤性两性因素有关，肌电图显示在静息时出现连续运动单位活动（CMUA）。与经典僵人综合征（SMS）不同，多表现为肢体远端强直及痛性痉挛，躯干相对较少累及；患者有多种抗体，糖尿病及血清抗谷氨酸脱羧酶抗体（GAD）较少见，个别患者患乳腺癌或肺小细胞癌；治疗效果不如经典SMS。结论 这是1例罕见的乳腺癌伴僵肢综合征的病例，根据临床特点，肌电图可以确诊，具有副肿瘤性及免疫学异常的特点，切除肿瘤后用大剂量激素及安全类药物治疗有效。

Stiff Limb Syndrome

Objective To study the clinicial, pathological, EMG features and therapy of stiff limb syndrome (SLS). Methods We reported the clinical manifestation, autoantibody, EMG, CSF and the prognosis in 1 patient with the limb rigidity and spasms in associated with breast cancer. The related literature were alao reviewed. Results Some patients had a paraneoplastic syndrome, and a non malignant autoimmune basis seemed likely in others. EMG presented continuous motor unit activity. Unlike the classic stiffman syndrome (SMS), the clinical picture is dominated by rigidity and painful spasms of the limbs, espically distally. There is a relative or total sparing in the trunk. Some autoantibodies were detected positively but the anti GAD was relatively rare. SLS was occasionally seen in the setting of breast or small cell lung carcinoma. The effects of therapy was poorer than that in classic SMS. Conclusions Stiff limb syndrome with breast cancer is rare, and clinical improvement can be with the treatment of the tumour, a large dose of prednisolone and diapazem. SLS is a sort of atypical SMS.