僵人综合征8例临床分析

An analysis of eight cases of stiff-person syndrome

目的分析8例僵人综合征(stiff-person syndrome,SPS)患者的病例资料,以提高对该病的早期诊断和治疗效果。方法回顾性分析8例SPS患者的临床表现、实验室检查结果、诊断和治疗经过,复习相关文献并探讨发病机制。结果 8例患者中亚急性发作1例,慢性发作7例。主要临床特点为四肢及躯干肌的僵直和痛性痉挛,其中3例患者分别累及舌肌、咽喉肌、面肌。7例给予巴氯芬及氯硝西泮可一定程度上缓解症状,1例拒绝进一步诊疗而自动出院。结论 SPS是一种罕见的自身免疫性疾病,具有不同的初始症状,发病后多累及四肢及躯干肌肉。苯二氮[艹卓]类和巴氯芬等可缓解症状。

Objective To analyze the clinical data of patients with stiff-person syndrome(SPS)and to review relevant literature as to improve the early diagnosis and treatment of the disease. Methods The clinical characteristics,laboratory examination,and diagnosis and treatment of 8 patients with SPS were analyzed retrospectively. The related literature was reviewed to discuss the pathogenesis. Results A total of 8 patients were analyzed,including 1 case of subacute onset and 7 cases of chronic onset. The main clinical features were stiffness and painful spasm of the limbs and trunk muscles,and 3 patients were involved in the tongue,throat muscles and facial muscles. In 7 cases,baclofen and clonazepam could relieve symptoms to some extent. One patient refused further medical treatment and discharged automatically. Conclusions SPS is a rare autoimmune disease with different initial symptoms. After the onset,it mainly involves the limbs and trunk muscles. Benzodiazepines and baclofen can alleviate the symptoms.