朊病毒病（Prion disease）又称海绵状脑病,是一组具有传染性和遗传性的神经变性性疾病。典型的病理变化局限在中枢神经系统,由神经细胞丢失、星形胶质细胞硬化、海绵状变性和淀粉斑所组成。以前我们曾对朊病毒病的致病因子朊病毒作过介绍,本文主要就其神经病理学进展及意义综述如下。1 神经元萎缩和星形胶质细胞硬化 Prion disease (Prion disease), also known as spongiform encephalopathy, is a group of infectious and hereditary neurodegenerative diseases. Typical pathological changes are confined to the central nervous system, consisting of loss of nerve cells, astrocytic sclerosis, spongiform degeneration and starch plaque. We have previously introduced prion pathogens prion disease, this article mainly on its neuropathological progress and its significance are summarized below. 1 Neuronal atrophy and astrocytic sclerosis