肌阵挛性小脑协调不良症(Dyssyner-gia cerebellaris myoclonica,Ramsay·HuntⅡ型综合征)是以动作性肌阵挛、小脑协调机能不良为特点并可伴有或不伴癫痫或Friedreich共济失调的综合征。本病是较少见的神经系统遗传性疾病,现将我院收治的2例报告如下。例1女,50岁,以左下肢走路不稳十年、左上肢不自主抖动四年入院。十年前一次骑自行车上街时突然发现左下肢无力、 Dyssyner-gia cerebellaris myoclonica (Ramsay Hunt type II syndrome) is characterized by action myoclonus, cerebellar coordination dysfunction with or without epilepsy or Friedreich’s ataxia Syndrome. The disease is a rare genetic disease of the nervous system, now admitted to our hospital 2 cases are as follows. Example 1 Female, 50 years old, with left lower extremity walking unsteady ten years, left upper limb involuntary jitter four years admitted. Ten years ago when riding a bicycle suddenly found the left limb weakness,