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。
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.
出处
《脑与神经疾病杂志》
2002年第2期69-71,共3页
Journal of Brain and Nervous Diseases
关键词
橄榄桥脑小脑萎缩
核磁共振
随访
olivopontocerebellar atrophy (OPCA)\ MRI\ follow up
