【摘要】：目的分析僵人綜合征的臨床特點、診治情況,提高對該病的早期診斷和治療。方法回顧性分析8例僵人綜合征患者的臨床表現(xiàn)、實驗室檢查、診治及隨訪情況,分析其病情變化。結果 8例患者主要表現(xiàn)為陣發(fā)性肌肉僵直和痛性痙攣。其中4例累及表情肌、舌肌、咀嚼肌、吞咽肌,眼球震顫2例,病理征陽性2例。血谷氨酸脫羧酶抗體陽性1例。住院期間8例均經(jīng)氯硝西泮、巴氯芬對癥治療,其中采用糖皮質(zhì)激素及丙種球蛋白免疫調(diào)節(jié)治療各1例,8例患者癥狀均緩解。成功隨訪了6例患者,隨訪期1個月至7年,其中1例出院后繼續(xù)糖皮質(zhì)激素和對癥治療半年后痊愈(隨訪7年),余5例出院后繼續(xù)采用對癥治療,其中3例癥狀無加重,2例預后不良(需推輪椅慢走1例,長期臥床1例)。結論僵人綜合征是一種罕見的自身免疫性疾病,早期診斷困難,易誤診。苯二氮艸卓類藥物、巴氯芬治療可有效控制癥狀。
[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.
【作者單位】： 湖南省人民醫(yī)院神經(jīng)內(nèi)科;中山大學附屬第一醫(yī)院神經(jīng)內(nèi)科;
【分類號】：R741

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