Global Brain Health Institute, University of California, San Francisco, 675 Nelson Rising Lane, Suite 190, San Francisco, CA 94518, USA; Department of Neurology, Buddhist Tzu Chi General Hospital, No. 707, Section 3, Zhong Yang Road, Hualien City, Hualien County 97002, Taiwan.
Global Brain Health Institute, University of California, San Francisco, 675 Nelson Rising Lane, Suite 190, San Francisco, CA 94518, USA; Dementia Department, National Institute of Neurology and Neurosurgery Manuel Velasco Suarez, Av. Insurgentes Sur 3877, Col. La Fama, Del. Tlalpan, Ciudad de México. C.P. 14269, Mexico.
Neurol Clin. 2018 Nov;36(4):865-897. doi: 10.1016/j.ncl.2018.07.005.
Prions diseases are uniformly fatal neurodegenerative diseases that occur in sporadic, genetic, and acquired forms. Acquired prion diseases, caused by infectious transmission, are least common. Most prion diseases are not infectious, but occur spontaneously through misfolding of normal prion proteins or genetic mutations in the prion protein gene. Although most prion diseases are not caused by infection, they can be transmitted accidentally. Certain infection control protocols should be applied when handling central nervous system and other high-risk tissues. New diagnostic methods are improving premortem and earlier diagnosis. Treatment trials have not shown improved survival, but therapies may be available soon.
朊病毒病是一种普遍致命的神经退行性疾病，有散发性、遗传性和获得性三种类型。获得性朊病毒病由传染性传播引起，最为罕见。大多数朊病毒病不具传染性，但通过正常朊蛋白的错误折叠或朊蛋白基因突变自发发生。虽然大多数朊病毒病不是由感染引起的，但它们可能会意外传播。在处理中枢神经系统和其他高风险组织时，应采用某些感染控制方案。新的诊断方法正在提高生前和更早的诊断水平。治疗试验并未显示生存率提高，但治疗方法可能很快就会出现。
