title: Ethics in prion disease.
abstract: 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% of prion diseases have a autosomal dominant genetic etiology, this further adds to the complexity of ethical issues, particularly regarding when to conduct genetic testing, release of genetic results, and when or if to implement experimental therapies. Human prion diseases are both infectious and transmissible; great care is required to balance the needs of the family and individual with both public health needs and strained hospital budgets. It is essential to proactively examine and address the ethical issues involved, as well as to define and in turn provide best standards of care.
authors.affiliation: Memory and Aging Center, University of California, San Francisco, United States.
authors.forename: Kendra
authors.initials: K
authors.lastname: Bechtel
authors.forename: Michael D
authors.initials: MD
authors.lastname: Geschwind
mesh_terms: D000818:Animals; D035843:Biomedical Research / Q000941:ethics*; D006801:Humans; D009462:Neurology / Q000941:ethics*; D017096:Prion Diseases / Q000175:diagnosis / Q000235:genetics / Q000628:therapy
keywords: 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–Sträussler–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
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