橄榄核-桥脑-小脑萎缩(Olivopontocerebellar atrophy)又称Dejerine-Ardre-Thomas综合征,是一类以累及小脑及其神经联系通路为主的变性疾病,常表现为一种多系统的损害,甚为少见。本病自1900年Dejerine和Thomas首先提出命名以来,国外迄今有文献记载的已百余例,绝大多数系个案报告。国内仅有1例临床病理报告。现将我们遇到的第1例尸检病例报告如下: Olivopontocerebellar atrophy, also known as Dejerine-Ardre-Thomas syndrome, is a type of degenerative disease involving the cerebellum and its neural pathway, which often manifests itself as a multi-system lesion, Rare. Since the disease was first proposed by Dejerine and Thomas in 1900, more than 100 foreign cases have been documented so far, the vast majority of which are reported on a case-by-case basis. Only one case of clinical pathology report. Now we encounter the first case of autopsy reported as follows: