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目的研究僵人综合征(stiff-personsyndrome,SPS;stiff-mansyndrome)及僵人叠加综合征(stiff-personplussyndrome,SPPS)的临床特点并观察静脉注射大剂量丙种球蛋白(IVIg)的近期治疗效果。方法回顾性分析1996—2005年我院6例SPS及SPPS的临床特点及4例IVIg(0·4g/kg×5d)治疗前后进行自身对照。结果5例女性(46±8)岁发病;1例新生儿(出生10d)发病,为我们所知目前发病最早的SPS,均缓慢起病,逐渐加重或波动性肌肉痉挛,颈肌、表情肌、咀嚼肌、咽部及呼吸肌多受累。眼肌受累3例,1例合并Chaddock征及Babinski征(+)。合并卵巢癌1例,糖尿病Ⅰ型1例。IVIg治疗难治性SPS4例,观察4~8个月,有较好疗效。结论(1)6例患者均为女性,1例病程22年的新生儿发病者,结合其他报道提示婴幼儿期发病者可能偏良性过程;(2)SPS作为一种自身免疫性疾病可在发现恶性肿瘤(如卵巢癌)数年前发病,此现象应引起高度重视;(3)IVIg治疗SPS近期疗效确实。
Objective To study the clinical features of stiff-person syndrome (SPS) and stiff-person complex syndrome (SPPS) and to observe the short-term therapeutic effect of intravenous injection of high-dose gamma globulin (IVIg). Methods The clinical features of 6 cases of SPS and SPPS in our hospital from 1996 to 2005 and 4 cases of IVIg (0.4g / kg × 5d) were retrospectively analyzed. Results A total of 5 women (46 ± 8) years old and 1 newborn (born 10 days old) were diagnosed as having the earliest onset of SPS. All of them were slow onset, gradually aggravating or fluctuating muscle spasms, cervical muscles, , Masticatory muscles, pharynx and respiratory muscle involvement. Eye muscle involvement in 3 cases, 1 case with Chaddock sign and Babinski sign (+). 1 case of ovarian cancer, type 1 diabetes mellitus. IVIg refractory treatment of SPS4 cases, observed 4 to 8 months, have a good effect. CONCLUSIONS: (1) All 6 patients were female. One case of newborns with a duration of 22 years, combined with other reports, suggested that infants and young children may be benign. (2) As an autoimmune disease, Malignant tumors (such as ovarian cancer) a few years ago, the incidence of this phenomenon should be attached great importance; (3) IVIg treatment of SPS recent efficacy indeed.