多系统萎缩的早期综合诊断

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。

Early Comprehensive Diagnosis in Multiple System Atrophy

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.