Referências

1. Prusiner, S.B., Novel proteinaceous infectious particles cause scrapie (1982) Science, 216, pp. 136-144
2. Prusiner, S.B., Prions (1998) Proc Natl Acad Sci USA, 95, pp. 13363-13383
3. Triarhou, L.C., Alfons Maria Jakob (1884-1931), neuropathologist par excellence: scientific endeavors in Europe and the Americas (2009) Eur Neurol, 61, pp. 52-58
4. Kanaani, J., Prusiner, S.B., Diacovo, J., Recombinant prion protein induces rapid polarization and development of synapses in embryonic rat hippocampal neurons in vitro (2005) J Neurochem, 95, pp. 1373-1386
5. Mallucci, G.R., White, M.D., Farmer, M., Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice (2007) Neuron, 53, pp. 325-335
6. van der Kamp, M.W., Daggett, V., Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding (2010) J Mol Biol, 404, pp. 732-748
7. Cobb, N.J., Surewicz, W.K., Prion diseases and their biochemical mechanisms (2009) Biochemistry, 48, pp. 2574-2585
8. Cali, I., Castellani, R., Yuan, J., Classification of sporadic Creutzfeldt-Jakob disease revisited (2006) Brain, 129, pp. 2266-2277
9. Mastrianni, J.A., The genetics of prion diseases (2010) Genet Med
10. Parchi, P., Giese, A., Capellari, S., Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects (1999) Ann Neurol, 46, pp. 224-233
11. Will, R.G., Ironside, J.W., Zeidler, M., A new variant of Creutzfeldt-Jakob disease in the UK (1996) Lancet, 347, pp. 921-925
12. Geschwind, M.D., Josephs, K.A., Parisi, J.E., Keegan, B.M., A 54-year-old man with slowness of movement and confusion (2007) Neurology, 69, pp. 1881-1887
13. Zerr, I., Kallenberg, K., Summers, D.M., Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease (2009) Brain, 132, pp. 2659-2668
14. Yemisci, M., Gurer, G., Saygi, S., Generalised periodic epileptiform discharges: clinical features, neuroradiological evaluation and prognosis in 37 adult patients (2003) Seizure, 12, pp. 465-472
15. Heath, C.A., Smith, C., Davenport, R., Progressive cognitive decline and myoclonus in a young woman: clinicopathological conference at the Edinburgh Advanced Neurology Course, 2007. Variant CJD. Subacute sclerosing panencephalitis (2008) Pract Neurol, 8, pp. 296-302
16. Korth, C., Peters, P.J., Emerging pharmacotherapies for Creutzfeldt-Jakob disease (2006) Arch Neurol, 63, pp. 497-501
17. Collinge, J., Gorham, M., Hudson, F., Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial (2009) Lancet Neurol, 8, pp. 334-344
18. Tsuboi, Y., Doh-Ura, K., Yamada, T., Continuous intraventricular infusion of pentosan polysulfate: clinical trial against prion diseases (2009) Neuropathology, 29, pp. 632-636
19. Bone, I., Belton, L., Walker, A.S., Intraventricular pentosan polysulphate in human prion diseases: an observational study in the UK (2008) Eur J Neurol, 15, pp. 458-464
20. Brown, S.A., Merritt, K., Woods, T.O., Effects on instruments of the World Health Organization: recommended protocols for decontamination after possible exposure to transmissible spongiform encephalopathy-contaminated tissue (2005) J Biomed Mater Res B Appl Biomater, 72, pp. 186-190
21. (2003) Practical Guidelines for Infection Control in Health Care Facilities, , Geneva
22. Sigurdson, C.J., A prion disease of cervids: chronic wasting disease (2008) Vet Res, 39, p. 41
23. Brown, P., Cathala, F., Castaigne, P., Gajdusek, D.C., Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases (1986) Ann Neurol, 20, pp. 597-602
24. Buganza, M., Ferrari, S., Cecchini, M.E., The oldest old Creutzfeldt-Jakob disease case (2009) J Neurol Neurosurg Psychiatry, 80, pp. 1140-1142
25. Berman, P.H., Davidson, G.S., Becker, L.E., Progressive neurological deterioration in a 14-year-old girl (1988) Pediatr Neurosci, 14, pp. 42-49
26. Gajdusek, D.C., Zigas, V., Degenerative disease of the central nervous system in New Guinea: the endemic occurrence of kuru in the native population (1957) N Engl J Med, 257, pp. 974-978
27. Will, R.G., Acquired prion disease: iatrogenic CJD, variant CJD, kuru (2003) Br Med Bull, 66, pp. 255-265
28. Collinge, J., Whitfield, J., McKintosh, E., Kuru in the 21st century: an acquired human prion disease with very long incubation periods (2006) Lancet, 367, pp. 2068-2074
29. Brown, P., Brandel, J.P., Preece, M., Sato, T., Iatrogenic Creutzfeldt-Jakob disease: the waning of an era (2006) Neurology, 67, pp. 389-393
30. Brandel, J.P., Preece, M., Brown, P., Distribution of codon 129 genotype in human growth hormone-treated CJD patients in France and the UK (2003) Lancet, 362, pp. 128-130
31. (2006) WHO guidelines on tissue infectivity distribution in transmissible spongiform encephalopathies. In: Quality and Safety of Plasma Derivatives and Related Substances Department of Medicines Policy and Standards Health Technology and Pharmaceuticals Cluster, , World Health Organization, pp 1-45
32. Koch, T.K., Berg, B.O., De Armond, S.J., Gravina, R.F., Creutzfeldt-Jakob disease in a young adult with idiopathic hypopituitarism. Possible relation to the administration of cadaveric human growth hormone (1985) N Engl J Med, 313, pp. 731-733
33. Boyd, A., Klug, G.M., Schonberger, L.B., Iatrogenic Creutzfeldt-Jakob disease in Australia: time to amend infection control measures for pituitary hormone recipients? (2010) Med J Aust, 193, pp. 366-369
34. Nombre de cas décédés certains ou probables de MCJ en France, , http://212.234.146.165/publications/mcj/donnees_mcj.html, Available at, Accessed Jun 2011
35. The National Creutzfeldt-Jakob Disease Research & Surveillance Unit, , http://www.cjd.ed.ac.uk, Available at, Accessed June. 2011
36. Billette de Villemeur, T., Deslys, J.P., Pradel, A., Creutzfeldt-Jakob disease from contaminated growth hormone extracts in France (1996) Neurology, 47, pp. 690-695
37. Healy, D.L., Evans, J., Creutzfeldt-Jakob disease after pituitary gonadotrophins (1993) BMJ, 307, pp. 517-518
38. Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts - Japan, January 1979-May 1996 (1997) MMWR Morb Mortal Wkly Rep, 46, pp. 1066-1069
39. Update: Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts - Japan, 1978-2008 (2008) MMWR Morb Mortal Wkly Rep, 57, pp. 1152-1154
40. Update: Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts - Japan, 1979-2003 (2003) MMWR Morb Mortal Wkly Rep, 52, pp. 1179-1181
41. Yamada, M., Noguchi-Shinohara, M., Hamaguchi, T., Dura mater graft-associated Creutzfeldt-Jakob disease in Japan: clinicopathological and molecular characterization of the two distinct subtypes (2009) Neuropathology, 29, pp. 609-618
42. Bernoulli, C., Siegfried, J., Baumgartner, G., Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery (1977) Lancet, 1, pp. 478-479
43. Will, R.G., Matthews, W.B., Evidence for case-to-case transmission of Creutzfeldt-Jakob disease (1982) J Neurol Neurosurg Psychiatry, 45, pp. 235-238
44. WHO tables on tissue infectivity distribution in transmissible spongiform encephalopathies, , http://www.who.int/bloodproducts/tablestissueinfectivity.pdf, Available at, Accessed June, 2011
45. Duffy, P., Wolf, J., Collins, G., Letter: Possible person-to-person transmission of Creutzfeldt-Jakob disease (1974) N Engl J Med, 290, pp. 692-693
46. Heckmann, J.G., Lang, C.J., Petruch, F., Transmission of Creutzfeldt-Jakob disease via a corneal transplant (1997) J Neurol Neurosurg Psychiatry, 63, pp. 388-390
47. Brown, P., Preece, M., Brandel, J.P., Iatrogenic Creutzfeldt-Jakob disease at the millennium (2000) Neurology, 55, pp. 1075-1081
48. Dorsey, K., Zou, S., Schonberger, L.B., Lack of evidence of transfusion transmission of Creutzfeldt-Jakob disease in a US surveillance study (2009) Transfusion, 49, pp. 977-984
49. European Creutzfeldt Jakob Disease Surveillance Network, , http://www.eurocjd.ed.ac.uk, Available at, Accessed June, 2011
50. Bovine spongiform encephalopathy (BSE), , http://www.cdc.gov/ncidod/dvrd/bse, Available at, Accessed June, 2011
51. Norrby, E., Prions and protein-folding diseases (2011) J Intern Med, 270, pp. 1-14
52. Heath, C.A., Cooper, S.A., Murray, K., Validation of diagnostic criteria for variant Creutzfeldt-Jakob disease (2010) Ann Neurol, 67, pp. 761-770
53. Heath, C.A., Cooper, S.A., Murray, K., Diagnosing variant Creutzfeldt-Jakob disease: a retrospective analysis of the first 150 cases in the UK (2011) J Neurol Neurosurg Psychiatry, 82, pp. 646-651
54. Rabinovici, G.D., Wang, P.N., Levin, J., First symptom in sporadic Creutzfeldt-Jakob disease (2006) Neurology, 66, pp. 286-287
55. Binelli, S., Agazzi, P., Giaccone, G., Periodic electroencephalogram complexes in a patient with variant Creutzfeldt-Jakob disease (2006) Ann Neurol, 59, pp. 423-427
56. Collie, D.A., Summers, D.M., Sellar, R.J., Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases (2003) AJNR Am J Neuroradiol, 24, pp. 1560-1569
57. Petzold, G.C., Westner, I., Bohner, G., False-positive pulvinar sign on MRI in sporadic Creutzfeldt-Jakob disease (2004) Neurology, 62, pp. 1235-1236
58. Will, R., Variant Creutzfeldt-Jakob disease (2004) Folia Neuropathol, 42, pp. 77-83
59. Budka, H., Neuropathology of prion diseases (2003) Br Med Bull, 66, pp. 121-130
60. Kaski, D., Mead, S., Hyare, H., Variant CJD in an individual heterozygous for PRNP codon 129 (2009) Lancet, 374, p. 2128
61. Fourth case of transfusion-associated variant-CJD infection (2007) Health Protection Report: weekly report, 1, pp. 2-3
62. Llewelyn, C.A., Hewitt, P.E., Knight, R.S., Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion (2004) Lancet, 363, pp. 417-421
63. Peden, A.H., Head, M.W., Ritchie, D.L., Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient (2004) Lancet, 364, pp. 527-529
64. Wroe, S.J., Pal, S., Siddique, D., Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report (2006) Lancet, 368, pp. 2061-2067
65. Peden, A., McCardle, L., Head, M.W., Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia (2010) Haemophilia, 16, pp. 296-304
66. Holada, K., Vostal, J.G., Theisen, P.W., Scrapie infectivity in hamster blood is not associated with platelets (2002) J Virol, 76, pp. 4649-4650
67. Garske, T., Ghani, A.C., Uncertainty in the tail of the variant Creutzfeldt-Jakob disease epidemic in the UK (2010) PLoS One, 5, p. e15626
68. Wallis, J.P., Strategies to reduce transfusion acquired vCJD (2011) Transfus Med, 21, pp. 1-6
69. Hilton, D.A., Ghani, A.C., Conyers, L., Accumulation of prion protein in tonsil and appendix: review of tissue samples (2002) BMJ, 325, pp. 633-634
70. Clewley, J.P., Kelly, C.M., Andrews, N., Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: cross sectional opportunistic survey (2009) BMJ, 338, p. b1442
71. de Marco, M.F., Linehan, J., Gill, O.N., Large-scale immunohistochemical examination for lymphoreticular prion protein in tonsil specimens collected in Britain (2010) J Pathol, 222, pp. 380-387
72. Hilton, D.A., Ghani, A.C., Conyers, L., Prevalence of lymphoreticular prion protein accumulation in UK tissue samples (2004) J Pathol, 203, pp. 733-739
73. Corato, M., Cereda, C., Cova, E., Young-onset CJD: age and disease phenotype in variant and sporadic forms (2006) Funct Neurol, 21, pp. 211-215
74. Hohler, A.D., Flynn, F.G., Onset of Creutzfeldt-Jakob disease mimicking an acute cerebrovascular event (2006) Neurology, 67, pp. 538-539
75. Murray, K., Ritchie, D.L., Bruce, M., Sporadic Creutzfeldt-Jakob disease in two adolescents (2008) J Neurol Neurosurg Psychiatry, 79, pp. 14-18
76. Monreal, J., Collins, G.H., Masters, C.L., Creutzfeldt-Jakob disease in an adolescent (1981) J Neurol Sci, 52, pp. 341-350
77. Brown, P., Cathala, F., Labauge, R., Epidemiologic implications of Creutzfeldt-Jakob disease in a 19 year-old girl (1985) Eur J Epidemiol, 1, pp. 42-47
78. Kulczycki, J., Jedrzejowska, H., Gajkowski, K., Creutzfeldt-Jakob disease in young people (1991) Eur J Epidemiol, 7, pp. 501-504
79. Meissner, B., Westner, I.M., Kallenberg, K., Sporadic Creutzfeldt-Jakob disease: clinical and diagnostic characteristics of the rare VV1 type (2005) Neurology, 65, pp. 1544-1550
80. Boesenberg, C., Schulz-Schaeffer, W.J., Meissner, B., Clinical course in young patients with sporadic Creutzfeldt-Jakob disease (2005) Ann Neurol, 58, pp. 533-543
81. Alperovitch, A., Zerr, I., Pocchiari, M., Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease (1999) Lancet, 353, pp. 1673-1674
82. Brown, K., Mastrianni, J.A., The prion diseases (2010) J Geriatr Psychiatry Neurol, 23, pp. 277-298
83. Oesch, B., Westaway, D., Walchli, M., A cellular gene encodes scrapie PrP 27-30 protein (1985) Cell, 40, pp. 735-746
84. Goldgaber, D., Goldfarb, L.G., Brown, P., Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Straussler-Scheinker's syndrome (1989) Exp Neurol, 106, pp. 204-206
85. Hsiao, K., Baker, H.F., Crow, T.J., Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome (1989) Nature, 338, pp. 342-345
86. Lloyd, S., Mead, S., Collinge, J., Genetics of prion disease (2011) Top Curr Chem, 305, pp. 1-22
87. Kovacs, G.G., Puopolo, M., Ladogana, A., Genetic prion disease: the EUROCJD experience (2005) Hum Genet, 118, pp. 166-174
88. Rogaeva, E., Zadikoff, C., Ponesse, J., Childhood onset in familial prion disease with a novel mutation in the PRNP gene (2006) Arch Neurol, 63, pp. 1016-1021
89. Rodriguez, M.M., Peoc'h, K., Haik, S., A novel mutation (G114V) in the prion protein gene in a family with inherited prion disease (2005) Neurology, 64, pp. 1455-1457
90. Kong, Q.K., Surewicz, W.K., Petersen, R.B., Inherited prion diseases (2004) Prion Biology and Disease, pp. 673-776. , Cold Spring Harbor Laboratory Press, Cold Spring Harbor, S.B. Prusiner (Ed.), 2nd ed
91. Venneti, S., Prion diseases (2010) Clin Lab Med, 30, pp. 293-309
92. Goldfarb, L.G., Petersen, R.B., Tabaton, M., Fatal familial insomnia and familial Creutzfeldt-Jakob disease: disease phenotype determined by a DNA polymorphism (1992) Science, 258, pp. 806-808
93. Hsich, G., Kenney, K., Gibbs, C.J., The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies (1996) N Engl J Med, 335, pp. 924-930
94. Sanchez-Juan, P., Green, A., Ladogana, A., CSF tests in the differential diagnosis of Creutzfeldt-Jakob disease (2006) Neurology, 67, pp. 637-643
95. Vitali, P., Maccagnano, E., Caverzasi, E., Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias (2011) Neurology, 76, pp. 1711-1719
96. Steinhoff, B.J., Zerr, I., Glatting, M., Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease (2004) Ann Neurol, 56, pp. 702-708
97. Seipelt, M., Zerr, I., Nau, R., Hashimoto's encephalitis as a differential diagnosis of Creutzfeldt-Jakob disease (1999) J Neurol Neurosurg Psychiatry, 66, pp. 172-176
98. Tschampa, H.J., Neumann, M., Zerr, I., Patients with Alzheimer's disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease (2001) J Neurol Neurosurg Psychiatry, 71, pp. 33-39
99. Shiga, Y., Miyazawa, K., Sato, S., Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jakob disease (2004) Neurology, 63, pp. 443-449
100. Young, G.S., Geschwind, M.D., Fischbein, N.J., Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis (2005) AJNR Am J Neuroradiol, 26, pp. 1551-1562
101. Geschwind, M.D., Shu, H., Haman, A., Rapidly progressive dementia (2008) Ann Neurol, 64, pp. 97-108
102. Hegde, A.N., Mohan, S., Lath, N., Lim, C.C., Differential diagnosis for bilateral abnormalities of the basal ganglia and thalamus (2011) Radiographics, 31, pp. 5-30
103. Karaarslan, E., Arslan, A., Diffusion weighted MR imaging in non-infarct lesions of the brain (2008) Eur J Radiol, 65, pp. 402-416
104. Global surveillance, diagnosis and therapy of human transmissible spongiform encephalopathies: report of a WHO consultation (1998) World Health Organization: Emerging and Other Communicable Diseases, Surveillance and Control, , 1998, 9-11 February
105. Geneva;