【摘要】 目的分析僵人综合征的临床特点、诊治情况,提高对该病的早期诊断和治疗。方法回顾性分析8例僵人综合征患者的临床表现、实验室检查、诊治及随访情况,分析其病情变化。结果 8例患者主要表现为阵发性肌肉僵直和痛性痉挛。其中4例累及表情肌、舌肌、咀嚼肌、吞咽肌,眼球震颤2例,病理征阳性2例。血谷氨酸脱羧酶抗体阳性1例。住院期间8例均经氯硝西泮、巴氯芬对症治疗,其中采用糖皮质激素及丙种球蛋白免疫调节治疗各1例,8例患者症状均缓解。成功随访了6例患者,随访期1个月至7年,其中1例出院后继续糖皮质激素和对症治疗半年后痊愈(随访7年),余5例出院后继续采用对症治疗,其中3例症状无加重,2例预后不良(需推轮椅慢走1例,长期卧床1例)。结论僵人综合征是一种罕见的自身免疫性疾病,早期诊断困难,易误诊。苯二氮艹卓类药物、巴氯芬治疗可有效控制症状。更多还原

【Abstract】 Objective To investigate the clinical features,diagnosis and treament of stiff-person syndrome(SPS),and to improve the early diagnosis and treatment of the disease. Methods The clinical manifestations,electrophysiological features,treatment and prognosis of 8cases with SPS were analyzed and the related literatures were also reviewed.Results The most commonly noticed symptoms were progressive or paroxysmal muscular rigidity and painful muscle spasm.Rigidity involved tongue muscles,expression muscles,chewing muscles and swallowing muscles in 4patients.Nystagmus was found in 2patients.Pyramidal signs were found in 2patients.Anti-glutamic acid decarboxylase(GAD)antibodies in serum were positive in 1patient.Symptoms of all case were relieved after clonazepam and baclofen treatment,while 1patient was treated with glucocorticoid and 1 patient was treated with intravenous immunoglobulin.6 patients were followed up successfully for 1to 84 months.1patient treated with glucocorticoid combined with clonazepam and baclofen after discharge had been cured 6months later(a follow-up period of 84months).The 5other patients continued to use symptomatic treatment with clonazepam and baclofen after discharge.Symptoms of 3patients remained unchanged by the drugsand condition of 2patients deteriorated(1patient walked by pushing wheelchair and 1patient lay in bed for a long period).Conclusions SPS is a rare autoimmune disease which is easy to be misdiagnosed at the early stage.Benzodiazepines and baclofen can help control symptoms,such as muscular rigidity or panic attack.更多还原