传染性海绵状脑病的诊断进展和存在的问题

传染性海绵状脑病是由朊病毒引起的人和多种哺乳动物以神经退行性变化为主要特征的一种慢性致死性传染病。引起这类疾病的病原因子是一种编码宿主蛋白的PrPC转变为异常的PrPSC沉积在大脑，导致传染性海绵状脑病的发生。本文从临床症状识别、组织病理学诊断、致病性朊蛋白检测、生物学测定以及毒株鉴定等几个方面作一回顾和总结，为揭示朊病毒疾病致病机理和诊断研究提供借鉴。

Progress and problems in the diagnostics of transmissible spongiform encephalopathy

The transmissible spongiform encephalopathies(TSEs),are a group of chronic fatal neurodegenerative diseases in humans and in a variety of mammalian animals.These prion diseases are associated with the deposition of PrPSc,a disease-specific isoform of the host-encoded cellular prion protein(PrPC),which accumulates in the brain affected with most forms of TSE.This article reviews and summarizes recent progress in the areas of the recognition of characteristic clinical signs,the diagnoses of histopathology,the detection of diseases-associated prion protein and the characteristic in biology,distinguish the strain of TSE,and expected it can use for reference of prion pathogenesis and diagnostics.