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This paper is intended to discuss some of the scientific and ethical issues that are created by increased research efforts towards earlier diagnosis, as well as to treatment of, human prion diseases (and related dementias), including the resulting consequences for individuals, their families, and society. Most patients with prion disease currently are diagnosed when they are about 2/3 of the way through their disease course (Geschwind et al., 2010a; Paterson et al., 2012b), when the disease has progressed so far that even treatments that stop the disease process would probably have little benefit. Although there are currently no treatments available for prion diseases, we and others have realized that we must diagnose patients earlier and with greater accuracy so that future treatments have hope of success. As approximately 15

作者:Kendra, Bechtel;Michael D, Geschwind

来源:Progress in neurobiology 2013 年 110卷

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作者:
Kendra, Bechtel;Michael D, Geschwind
来源:
Progress in neurobiology 2013 年 110卷
标签:
BSE Bovine Spongiform Encephalopathy CDC CJD CSF CWD Centers for Disease Control Chronic Wasting Disease Creutzfeldt-Jakob Disease DWI Diffusion Weighted imaging Ethics FDA FFI FLAIR Familial CJD Fatal Familial Insomnia Fluid Attenduated Inversion Recovery Food and Drug Administration GINA GSS Genetic Information Nondiscrimination Act Genetic Prion Disease Gerstmann–Str?ussler–Scheinker HD Huntington's Disease Iatrogenic CJD NGT NSE Nasogastric Tube Neurodegenerative disorders Neuron Specific Enolase OPRI Octopeptide Repeat Insertion PGD PPS Pentosan Polysulphate Preimplantation Genetic Diagnosis Prion Prion disease RPD Rapidly Progressive Dementia Sporadic CJD TSE Transmissible Spongiform Encephalopathy University of California, San Francisco Variant CJD WHO World Health Organization fCJD gPrD iCJD sCJD vCJD
This paper is intended to discuss some of the scientific and ethical issues that are created by increased research efforts towards earlier diagnosis, as well as to treatment of, human prion diseases (and related dementias), including the resulting consequences for individuals, their families, and society. Most patients with prion disease currently are diagnosed when they are about 2/3 of the way through their disease course (Geschwind et al., 2010a; Paterson et al., 2012b), when the disease has progressed so far that even treatments that stop the disease process would probably have little benefit. Although there are currently no treatments available for prion diseases, we and others have realized that we must diagnose patients earlier and with greater accuracy so that future treatments have hope of success. As approximately 15