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A deadly prion disease: fatal familial insomnia.
J Neurosci Nurs. 2003 Dec; 35(6):300-5.JN

Abstract

Fatal familial insomnia (FFI) is an inherited disease caused by a mutation in the protein prion gene. Symptoms of FFI closely resemble those of familial Creutzfeldt-Jakob disease, making genetic testing and histological examination of brain tissue the only means to determine a definitive diagnosis. The disease is rare--approximately 60 cases have been detected worldwide since 1986. Incubation time of the disease may be as long as 30 years; death generally occurs within 1 year of the onset of symptoms. There is no known procedure or treatment for delaying the onset of symptoms or modifying the disease course. Nurses who confront patients with FFI will be challenged to provide care to a patient and family who are facing certain death.

Authors+Show Affiliations

University of Pennsylvania, USA.No affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

14713095

Citation

Sundstrom, Dianne G., and H Michael Dreher. "A Deadly Prion Disease: Fatal Familial Insomnia." The Journal of Neuroscience Nursing : Journal of the American Association of Neuroscience Nurses, vol. 35, no. 6, 2003, pp. 300-5.
Sundstrom DG, Dreher HM. A deadly prion disease: fatal familial insomnia. J Neurosci Nurs. 2003;35(6):300-5.
Sundstrom, D. G., & Dreher, H. M. (2003). A deadly prion disease: fatal familial insomnia. The Journal of Neuroscience Nursing : Journal of the American Association of Neuroscience Nurses, 35(6), 300-5.
Sundstrom DG, Dreher HM. A Deadly Prion Disease: Fatal Familial Insomnia. J Neurosci Nurs. 2003;35(6):300-5. PubMed PMID: 14713095.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A deadly prion disease: fatal familial insomnia. AU - Sundstrom,Dianne G, AU - Dreher,H Michael, PY - 2004/1/10/pubmed PY - 2004/3/12/medline PY - 2004/1/10/entrez SP - 300 EP - 5 JF - The Journal of neuroscience nursing : journal of the American Association of Neuroscience Nurses JO - J Neurosci Nurs VL - 35 IS - 6 N2 - Fatal familial insomnia (FFI) is an inherited disease caused by a mutation in the protein prion gene. Symptoms of FFI closely resemble those of familial Creutzfeldt-Jakob disease, making genetic testing and histological examination of brain tissue the only means to determine a definitive diagnosis. The disease is rare--approximately 60 cases have been detected worldwide since 1986. Incubation time of the disease may be as long as 30 years; death generally occurs within 1 year of the onset of symptoms. There is no known procedure or treatment for delaying the onset of symptoms or modifying the disease course. Nurses who confront patients with FFI will be challenged to provide care to a patient and family who are facing certain death. SN - 0888-0395 UR - https://wwww.unboundmedicine.com/medline/citation/14713095/A_deadly_prion_disease:_fatal_familial_insomnia.&sa=D&ust=1576193332742000 L2 - http://ovidsp.ovid.com/ovidweb.cgi?T=JS&PAGE=linkout&SEARCH=14713095.ui DB - PRIME DP - Unbound Medicine ER -