Shy-Drager综合征少见。本文报告16例临床资料。男12例，女4例，以47～60岁居多（14例）。都有典型的小脑症状（眼震、构音障碍和共济失调）、体位性低血压及排尿淋漓及男性阳萎植物神经功能障碍。部分尚有锥体外系和锥体束征。CT／MRI扫描显示小脑和脑干萎缩。本症缺乏有效的治疗方法，合理提高血压可预防体位性低血压晕厥。 Shy-Drager syndrome rare. This article reports 16 cases of clinical data. There were 12 males and 4 females, mostly 47 to 60 years old (14 cases). Have a typical cerebellar symptoms (nystagmus, dysarthria and ataxia), orthostatic hypotension and urination dripping and male patients with impaired autonomic dysfunction. Some still have extrapyramidal system and pyramidal tract signs. CT / MRI scan showed cerebellar and brainstem atrophy. The lack of effective treatment of this disease, a reasonable increase in blood pressure can prevent orthostatic hypotension syncope.