35例多系统萎缩的临床及实验室检查特点分析

The clinical and laboratory profile of multiple system atrophy:analysis of 35 patients

目的 分析多系统萎缩(MSA)的临床及实验室检查特点.方法 回顾性分析 35 例多系统萎缩患者的临床表现、辅助检查和治疗情况.患者中男 26 例,女 9 例,病程平均 48 个月(5 个月至 12 年).结果 患者起病年龄 21～72 岁,88%为40岁以后发病,男多于女(2.8∶1.0).其中橄榄体桥小脑萎缩(OPCA)26例,22(84%)例首发症状为走路不稳.Shy-Drager 氏综合征(SDS)7例,均以直立性头晕为首发症状.纹状体-黑质变性(SND)2例,首先表现为运动减少.在所有患者中,小脑体征以共济失调为主(88%),其他有眼震、爆发言语;植物神经障碍主要为便秘(88%)、排尿障碍(83%)、阳萎(80%),体位性低血压(20%),少见者有低热(2例),Horner 氏综合征(1例);锥体外系症状以表情少、运动减少为主(57%),其次为肌张力增高(30%),震颤相对少见(11%).其他尚有锥体束征、脑干症状,周围神经体征.MRI检查,所有受检查的OPCA患者(15例)见小脑、脑桥、橄榄体萎缩,严重者四脑室、枕大池、桥前池增大,而大脑萎缩轻,三脑室、侧脑室扩大少见.SDS患者和SND患者MRI缺乏特异性.47%患者脑干听觉诱发电位(BAEP)异常.37%的患者视诱发电位(VEP)和40%的患者体感诱发电位(SEP)异常.脑电图、脑脊液检查基本正常.经治疗,5例患者症状好转.结论 MSA是一组神经系统多部位变性综合征,各型早期各具特点,随病情进展,出现其他系统症状体征.MRI等有关检测技术对诊断有肯定的意义.目前无治本药物,但有些药物可改善部分患者临床症状.

Objective To analysis the clinical and laboratory features of multiple system atropy(MSA).Methods The clinical and laboratory data of 35 patients with MSA,26 males and 9 females,were retrospectively analyzed.Results Among the 35 patients,26 cases were classified as olivopontocerebellar atrophy(OPCA),7 as Shy-Drager syndrome(SDS),and 2 as striatonigral degeneration(SND).Most patients developed symptoms after 40 years old.The incidence was higher in males than that in females.The initial symptom of 22 out of the 26 patients with OPCA(84%)was cerebella ataxia.All 7 patients with SDS complained of dizziness while standing up.The patients with SND showed extra-pyramidal symptoms as the initial symptoms.Other systems often occurred within 3 years.The cranial MRI of patients with OPCA showed obvious atrophy in olive-pons and cerebella.However,MRI in the patients with SDS and SND showed no special features.Brainstem auditoy evoked potentials(BAEP)revealed abnormality in 47% cases.Electroencephalogram(EEG)and cerebrospinal fluid(CSF)were generally normal.Some patients benefited from treatment.Conclusion MSA is a degenerative disorder involving in several nerve systems.The clinical features differ with types.Neuroimaging and other laboratory examinations are helpful for making diagnoses.Although there is no radical treatment,certain medicines do make improvement in some patients.