所谓朊病毒病是指由朊病毒引起的，具有新型复制和传播致病因子的一系列神经变性性疾病。朊病毒病以潜伏期长达数月到数年、一旦发病不可逆转为特征。这种病还被称作为可传播的海绵状脑病，因为可以在中枢神经系统内观察到显著的神经病理改变。在这类疾病中最重要的成员是男性偶发遗传性和感染性克雅氏病（CJD），牛海绵状脑病（BSE，疯牛病）和羊瘙痒病。虽然这些疾病很罕见，但是人类朊病毒病最近被媒体广泛报道，因为很有可能在人类变异型克雅氏病和牛海绵状脑病之间存在着联系，而且有可能人血和血液制品会被变异型克雅氏病致病因子污染。这一简短的综述讨论了朊病毒的基础生物学特征，并讨论了最重要的人类朊病毒病的临床表现和病理学特征。

PMID: 11087170, UI: 20538005
Human prion diseases.

Haltia M

Department of Pathology, University of Helsinki and the Helsinki University Central Hospital, Finland. matti.j.haltia@helsinki.fi

[Medline record in process]

The term 'prion diseases' refers to a group of neurodegenerative disorders thought to be caused by prions, pathogenic agents with novel modes of replication and transmission. Prion diseases are characterized by long incubation periods ranging from months to years and are invariably fatal once clinical symptoms have appeared. They are also called transmissible spongiform encephalopathies (TSE), on account of the predominant neuropathological change observed in the central nervous system. The most important members of this group are Creutzfeldt-Jakob disease (CJD) of man displaying sporadic, inherited and infectious forms, bovine spongiform encephalopathy (BSE, 'mad cow disease') of cattle, and scrapie of sheep. Despite their rarity, human prion diseases have recently been covered extensively in the media because of the likely connection between a new variant of human CJD (vCJD) and BSE and the possibility of contamination of human blood and blood products by the vCJD agent. This short review discusses the basic biological properties of prions, followed by a presentation of the clinical and pathological features of the most important human prion diseases.

PMID: 11087170, UI: 20538005