Publicado Nov 12, 2013



PLUMX
Almetrics
 
Dimensions
 

Google Scholar
 
Search GoogleScholar
Descargas


Nimrod Shabtai Barashi Gozal

Catalina Vargas Acevedo

Luis Alfonso Zarco

##plugins.themes.bootstrap3.article.details##

Resumen

Los priones son partículas proteicas que, tras una alteración en su estructura secundaria en un proceso postraducción, se vuelven patógenos infecciosos y son responsables de las encefalopatías espongiformes transmisibles (EET). La EET más común en humanos es la enfermedad de Creutzfeldt-Jakob (ECJ) que, según su causa y sus características clínico-patológicas, puede dividirse en cuatro formas: esporádica, genética, iatrogénica y variante. Esta enfermedad se manifiesta como una demencia rápidamente progresiva en personas de mediana edad y ancianos; presenta una incidencia anual de aproximadamente un caso por millón de habitantes. En el presente artículo se realizará una revisión sistemática de la literatura basada en artículos publicados en los últimos diez años acerca de la ECJ, utilizando las bases de datos MedLine y Embase.

Keywords

prion, síndrome de Creuztfeldt-Jakob, demencia, Prion, Creutzfeldt-Jakob syndrome, dementia,

References
1. Hofmann J, Wolf H, Grassmann A, Arndt V, Graham J, Vorberga I. Creutzfeldt-Jakob disease and mad cows: lessons learnt from yeast cells. Swiss Med Wkly. 2012 Jan 24;142:w13505. doi: 10.4414/smw.2012.13505.
2. Cohen OS, Prohovnik I, Korczyn AD, Inzelberg R, Nitsan Z, Appel S, et al. Characterization of movement disorders in patients with familial Creutzfeldt-Jakob disease carrying the E200K mutation. Isr Med Assoc J. 2012;14(3):162-5.
3. Norrby E. Prions and protein-folding diseases. J Intern Med. 2011;270:1-14.
4. Ryou C. Prions and prion diseases: fundamentals and mechanistic details. J Microbiol Biotechnol. 2007;17(7):1059-70.
5. Haïk S, Brandel J-P. Biochemical and strain properties of CJD prions: Complexity versus simplicity. J Neurochem. 2011;119:251-61.
6. Torres M, Cartier L, Matamala JM, Hernández N, Woehlbier U, Hetz C. Altered prion protein expression pattern in CSF as a biomarker for Creutzfeldt-Jakob disease. PLoS One.2012;7(4).
7. Ironside JW. Variant Creutzfeldt-Jakob Disease. Haemophilia. 2010;16:175-80.
8. Puoti G, Bizzi A, Forloni G, Safar JG, Tagliavini F, Gambetti P. Sporadic human prion diseases: molecular insights and diagnosis. Lancet Neurol. 2012;11:618-28.
9. Safar JG. Molecular pathogenesis of sporadic prion diseases in man. Prion. 2012;6(2):108-15.
10. Oxford University Press on Behalf of the Guarantors of Brain. Sporadic Creutzfeldt-Jakob disease: discrete subtypes or a spectrum of disease? Brain. 2009;132:2627-9.
11. Hill AF, et al. Molecular classification of sporadic Creutzfeldt-akob disease. Brain. 2003;126:1333-46.
12. Oxford University Press on Behalf of the Guarantors of Brain. Sporadic Creutzfeldt-Jakob disease: further twists and turns in a convoluted protein. Brain. 2006;129:2238-40.
13. Sanchez-Juan P, Bishop MT, Croes EA, Knight RS, Will RG, van Duijn CM, Manson JC. A polymorphism in the regulatory region of PRNP is associated with increased risk of sporadic Creutzfeldt-Jakob disease. BMC Med Genet. 2011;12(73):1-6.
14. Imran M, Mahmood S. An overview of human prion diseases. Virol J. 2001;8:559.
15. Parchi P, Cescatti M, Notari S, Schulz-Schaeffer WJ, Capellari S, Giese A. Agent strain variation in human prion disease: insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease. Brain. 2010;133:3030-42.
16. Knight RSG, Will RG. Prion diseases. J Neurol Neurosurg Psychiatry. 2004;75:i36-42.
17. Knight R. Creutzfeldt-Jakob disease: a rare cause of dementia in elderly persons. Clin Infect Dis. 2006 Aug 1;43(3):340-6.
18. Brown K, Mastrianni JA. The prion diseases. J Geriatr Psychiatry Neurol. 2010;23(4):277-98.
19. Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto A, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain. 2009;132(Pt 10):2659-68.
20. Krasnianski A, Schulz-Schaeffer WJ, Kallenberg K, Meissner B, Collie DA, Roeber S, et al. Clinical findings and diagnostic tests in the MV2 subtype of sporadic CJD. Brain. 2006;129:2288-96.
21. Macfarlane RG, Wroe SJ, Collinge J, Yousry TA, Jäger HR. Neuroimaging findings in human prion disease. J Neurol Neurosurg Psychiatry. 2007;78:664-70.
22. Riva-Amarante E, Jiménez-Huete A, Toledano R, Calero M, Alvarez-Linera J, Escribano J, et al. Usefulness of high b-value diffusion-weighted MRI in the diagnosis of Creutzfeldt-Jakob disease. Neurología. 2011;26(6):331-6.
23. Zhang WJ, Westover MB, Keary CJ. Premortem diagnosis of sporadic Creutzfeldt-Jakob disease aided by positron-emission tomography imaging. AJNR Am J Neuroradiol. 2011 Jan;32(1):E18. doi: 10.3174/ajnr.A2292.
24. Ladogana A, Sanchez-Juan P, Mitrová E, Green A, Cuadrado-Corrales N, Sánchez-Valle R, et al. Cerebrospinal fluid biomarkers in human genetic transmissible spongiform encephalopathies. J Neurol. 2009;256(10):1620-28.
25. Muayqil T, Gronseth G, Camicioli R. Evidence-based guideline: Diagnostic accuracy of CSF 14-3-3 protein in sporadic Creutzfeldt-Jakob disease: report of the Guideline Development Subcommittee of the American Academy of Neurology. Neurology. 2012;79.
26. Oeckl P, Steinacker P, Lehnert S, Jesse S, Kretzschmar HA, Ludolph AC, et al. CSF concentrations of cAMP and cGMP are lower in patients with Creutzfeldt-Jakob disease but not Parkinson’s disease and amyotrophic lateral sclerosis. PLoS One. 2012;7(3).
27. Singh A, Beveridge AJ, Singh N. Decreased CSF transferrin in sCJD: a potential pre-mortem diagnostic test for prion disorders. PLos One. 2011;6(3):1-10.
Cómo citar
Barashi Gozal, N. S., Vargas Acevedo, C., & Alfonso Zarco, L. (2013). Enfermedades priónicas humanas. Universitas Medica, 54(4), 495–516. https://doi.org/10.11144/Javeriana.umed54-4.ephu
Sección
Artículos de revisión

Artículos más leídos del mismo autor/a