僵人综合征8例诊疗分析

发布时间：2019-08-13 18:11

【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。
[Abstract]:Objective to analyze the clinical characteristics, diagnosis and treatment of rigid syndrome, and to improve the early diagnosis and treatment of the syndrome. Methods the clinical manifestations, laboratory examination, diagnosis, treatment and follow-up of 8 patients with rigid syndrome were analyzed retrospectively. Results the main manifestations of 8 patients were paroxysmal muscle stiffness and painful spasm. Among them, 4 cases involved emoji muscle, tongue muscle, masticatory muscle, swallowing muscle, nystagmus in 2 cases, pathological sign positive in 2 cases. Blood glutamic acid decarboxylase antibody was positive in 1 case. During hospitalization, 8 cases were treated with clonazepam and baclofenac, of which 1 case was treated with glucocorticoid and 1 case with gamma globulin immunomodulatory therapy, and 8 cases were relieved. Six patients were followed up successfully for a period of 1 month to 7 years. One of them recovered from glucocorticoid and symptomatic treatment for half a year after discharge (follow-up for 7 years). The other 5 patients continued to be treated with symptomatic treatment after discharge. Among them, 3 cases had no aggravation of symptoms, 2 cases had poor prognosis (1 case needed to push wheelchair slowly and 1 case stayed in bed for a long time). Conclusion rigid person syndrome is a rare autoimmune disease, which is difficult to diagnose early and easy to be misdiagnosed. Benzodiazepine drugs, baclofen treatment can effectively control symptoms.
【作者单位】：湖南省人民医院神经内科;中山大学附属第一医院神经内科;
【分类号】：R741

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