【摘要】 目的 :探讨橄榄桥脑小脑萎缩 (OPCA)患者病情的发生、发展和转归的规律及可能影响因素。方法 :对确诊的 38例 (家族性 2例 ,散发性 36例 )进行脑脊液、头颈 CT、 MRI检查及临床特点分析 ,并对其中 2 8例进行了 1～ 12年随访。结果 :1SOPCA和 FOPCA平均发病年龄分别为 4 6 .9岁和 2 0岁 ;平均病程分别为 6 .7年和 14 .5年。 2受累系统症状出现频率为小脑共济失调 92 .1% ,锥体束征 86 .8% ,构音障碍 84 .2 % ,自主神经损害 76 .3%。 3病程第一年内头颅 CT和 MRI分别有 16 .7%和 70 %的异常。 4平均确诊时间为 4 .1年 ,目前治疗无法改变其转归 ,随访 2 8例死亡 3例 ,死因分别为吸入性肺炎、窒息及全身衰竭。结论 :OPCA受累系统以小脑最多 ,无遗传家族史者病情发展较快 ,多死于并发症。核磁共振为首选辅助检查 ;随访有助于早期确诊。更多还原

【Abstract】 Objective: To investigate the orderliness and the affective factors of the onset, developing and prognosis in the patients with olivopontocerebellar atrophy (OPCA). Methods: We analyzed the CSF, CT MRI and the clinical manifestation of 38 cases (2 cases were family OPCA, 36 cases were sporadic OPCA) who were definitely diagnosed. And we make a follow up of 1~12 years. Results: ①The average ages of onset of SOPCA and FOPCA are 46.9 years and 20 years respectively. The average course is 6.7 years and 14.5 years respectively. ②The frequency of cerebellar ataxia, pyramidal bame signs, dysarthria and damages of automatic nervous system are 92.1%, 86.8%, 84.2% and 76.3 % respectively. ③During the first year after onset, there are 16.7% of patients with abnormal CT and 70% with abnormal MRI. ④The average time of definite diagnosis is 4.1 years. The present treatments can’t change the prognosis. There are 3 cases dead of inhaling pneumonia, suffocation and dyscrasia during the follow up. Conclusion: The cerebellar symptoms of OPCA are more severe and early than others. FOPCA progress more slowly than SOPCA. MRI and the follow up will be halpful for the early diagnosis.更多还原