23例多系统萎缩的临床与影像学分析

目的：探讨多系统萎缩（ＭＳＡ）的影像学特征与临床表现的相关性，为临床诊断提供依据。方法：对２３例临床资料进行回顾性分析。结果：根据临床资料及影像结果分为３类型。（１）１６例橄榄体桥小脑萎缩（ＯＰＣＡ）中，以小脑共济失调为主者１０例，脑的ＣＴ或ＭＲＩ可见桥脑、橄榄体、小脑萎缩明显，可伴大脑等部位萎缩；（２）３例Ｓｈｙ－Ｄｒａｇｅｒ综合征（ＳＤＳ），以植物神经功能紊乱为主要表现者２例，１例误诊为多发脑梗塞，脑ＭＲＩ壳核Ｔ２低信号改变；（３）４例纹状体－黑质变性（ＳＮＤ）首发症状均为锥体外系表现而误诊为帕金森病。脑ＭＲＩ示壳核可有Ｔ１低Ｔ２高信号改变。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，其中ＳＤＳ、ＳＮＤ较易误诊；ＭＲＩ等有关检测技术对发病机理和诊断分型等方面有较肯定的意义

Clinical and neuroimage features of multiple system atrophy:Analysis of 23 cases

objective:To probe into the correlation between clinical features and neuroimage of multiple system atrophy (MSA) and to provide evidence for making clinical diagnoses.Methods:The clinical data of 23 patients with MSA were retrospectively analysed and discussed.Resluts:Three types were classified according to the clinical materials and the results of neuroimage:(1) Olivopontocerebellar atrophy (OPCA):10 out of 16 patients with OPCA presented chiefly as cerebellar ataxia.The cranial MRI or CT of patients with OPCA showed obvious atrophy in olivopons and cerebella,and cerebral atrophy occured in some patients;(2)Shy Drager Syndrome (SDS):2 out of 3 patients with SDS presented chiefl as autonomic nervous disturbance and 1 was misdiagnosed as multiple cerebral infarction.Low intensity T 2 signals on Cranial MRI of patients with SDS were detected in strionigral areas;(3)striatonigral degeneration(SND):Because 4 patients with SND occurred at first the extrapyramid nal symptoms,they were misdiagnosed as Parkinson disease in early stage of disease.Cranial MRI of SND showed that a low intensity T1 and high intensity T 2 signal in trionigral areas.Conclusions:MSA is a symptom of multiple areas degeneration of nervous system.The clinical features differ with various types. In early stage of the disease,SDS and SND are usually misdiagnosed.Neuroimage examinations are helpful for making diagnoses and determinng pathogenesis of MSA.