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目的：探讨多系统萎缩(multiple system atrophy，MSA)患者的临床表现及神经影像学新特征(脑桥“十字征”和“壳核裂隙征”) 在MSA早期诊断中的临床意义。方法：回顾性分析21 例临床诊断为多系统萎缩（MSA）患者的临床表现和头部MRI资料。结果：21例MSA患者中，Shy-Drager综合征（MSA-A）9例，早期临床表现为体位性低血压，泌尿生殖功能障碍，头部MRI检查脑桥 “壳核裂隙征”和“十字征”为Ⅰ期；橄榄体脑桥小脑萎缩(MSA-C)5 例，3 例发病后1 年头部MRI脑桥“十字征”达Ⅱ期；“壳核裂隙征”为Ⅰ期，2例发病后3年头部MRI脑桥“十字征”达Ⅳ期。黑质纹状体变性(MSA-P)7例：早期临床均有运动迟缓、震颤等表现， 3例发病后1年脑桥“十字征”Ⅰ期，“壳核裂隙征”Ⅲ期；3 例发病后2 年脑桥“十字征”Ⅰ期，“壳核裂隙征”Ⅰ期；另1 例发病后9 月脑桥“十字征”Ⅰ期，“壳核裂隙征”Ⅱ期。结论：认为临床表现与头部MRI检查显示的新的影像学特征结合有助于MSA 早期诊断。

英文摘要:

Objective: To analyze the clinical manifestations as well as correlation between clinical manifestations and the new neuroimage characteristics (pontine"cross sign"and "putaminal slit")for early diagnosis of MSA. Methods:21 patients with diagnostic as multiple system atrophy (MSA) were retrospectively an analyzed for clinical manifestations and brain MRI findings．Results:In 21 patients with MSA，9 patients were MSA-A, eariy clinical manifestations of orthostatic hypotension,urogenital dysfunction, MRI showed stage Ⅰ"Cross sign "and" putaminal slit". 5 patients were MSA-C, early clinical appear unstable walking,ataxia and other performance, MRI showed stage Ⅱ"Cross sign "and stage Ⅰ" putaminal slit"after onset.7 patients were MSA-P,early clinical are bradykinesia,tremor and other performance,3 patients reached stage Ⅰ"Cross sign "and stage Ⅳ" putaminal slit" one years after onset, 3 patient reached stage Ⅰ"Cross sign "and stage Ⅰ" putaminal slit"two years after onset, another patient reached stage Ⅰ"Cross sign "and stage Ⅱ" putaminal slit" nine months after onset. Conclusions:Clinical manifestations and the new neuroimage characteristics were very important for early diagnosis of MSA.

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