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Vol. 37, No. 3-4, 2011
Issue release date: December 2011

Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance? A Study in France from 2000 to 2008

Brandel J.-P. · Welaratne A. · Salomon D. · Capek I. · Vaillant V. · Aouaba A. · Haïk S. · Alpérovitch A.
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Background: Surveillance of Creutzfeldt-Jakob disease (CJD) is still an important issue because of the variant CJD epidemic, which is in decline and also because of the emergence of novel forms of animal transmissible spongiform encephalopathy with zoonotic potential and the risk of nosocomial and blood transfusion-related transmission. Active surveillance has been implemented in most European countries and requires important human resources and funding. Here, we studied whether national mortality and morbidity statistics can be used as reliable indicators. Methods: CJD data collected by the French national CJD surveillance centre were compared with data registered in the national mortality statistics. Results: From 2000 to 2008, the two sources reported fairly similar numbers of CJD deaths. However, analysis of individual data showed important between-sources disagreement. Nearly 24% of CJD reported by the mortality register were false-positive diagnoses and 21.6% of the CJD cases diagnosed by the surveillance centre were not registered as CJD in the national mortality statistics. One out of 22 variant CJD cases was not reported as having any type of CJD in the mortality statistics. Conclusions:These findings raise doubt about the possibility of a reliable CJD surveillance only based on mortality data.

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  1. Bertrand A, Brandel JP, Grignon Y, Sazdovitch V, Seilhean D, Faucheux B, Privat N, Brault JL, Vital A, Uro-Coste E, Pluot M, Chapon F, Maurage CA, Letournel F, Vespignani H, Place G, Degos CF, Peoc’h K, Haïk S, Hauw JJ: Wernicke encephalopathy and Creutzfeldt-Jakob disease. J Neurol 2009;256:904–909.
  2. Peoc’h K, Delasnerie-Lauprêtre N, Beaudry P, Laplanche JL: Diagnostic value of CSF 14-3-3 detection in sporadic CJD diagnosis according to the age of the patient. Eur J Neurol 2006;13:427–428.
  3. Puopolo M, Ladogana A, Vetrugno V, Pocchiari M: Transmission of sporadic Creutzfeldt-Jakob disease by blood transfusion: risk factor or possible biases. Transfusion 2011;51:1556–1566.
  4. de Pedro-Cuesta J, Mahillo-Fernández I, Rábano A, Calero M, Cruz M, Siden A, Laursen H, Falkenhorst G, Mølbak K; EUROSURGYCJD Research Group: Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from a risk-based assessment of surgical interventions. J Neurol Neurosurg Psychiatry 2011;82:204–212.
  5. Mahillo-Fernandez I, de Pedro-Cuesta J, Bleda MJ, Cruz M, Mølbak K, Laursen H, Falkenhorst G, Martínez-Martín P, Siden A; EUROSURGYCJD Research Group: Surgery and risk of sporadic Creutzfeldt-Jakob disease in Denmark and Sweden: registry-based case-control studies. Neuroepidemiology 2008;31:229–240.
  6. Ward HJ, Everington D, Cousens SN, Smith-Bathgate B, Gillies M, Murray K, Knight RS, Smith PG, Will RG: Risk factors for sporadic Creutzfeldt-Jakob disease. Ann Neurol 2008;63:347–354.
  7. Garske T, Ghani AC: Uncertainty in the tail of the variant Creutzfeldt-Jakob disease epidemic in the UK. PLoS One 2010;5:e15626.
  8. Sandberg MK, Al-Doujaily H, Sigurdson CJ, Glatzel M, O’Malley C, Powell C, Asante EA, Linehan JM, Brandner S, Wadsworth JD, Collinge J: Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein. J Gen Virol 2010;91:2651–2657.
  9. Mawhinney S, Pape WJ, Forster JE, Anderson CA, Bosque P, Miller MW: Human prion disease and relative risk associated with chronic wasting disease. Emerg Infect Dis 2006;12:1527–1535. Erratum in: Emerg Infect Dis 2006;12:1805.
  10. Comoy EE, Casalone C, Lescoutra-Etchegaray N, Zanusso G, Freire S, Marcé D, Auvré F, Ruchoux MM, Ferrari S, Monaco S, Salès N, Caramelli M, Leboulch P, Brown P, Lasmézas CI, Deslys JP: Atypical BSE (BASE) transmitted from asymptomatic aging cattle to a primate. PLoS One 2008;3:e3017.
  11. Casalone C, Zanusso G, Acutis P, Ferrari S, Capucci L, Tagliavini F, Monaco S, Caramelli M: Identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic Creutzfeldt-Jakob disease. Proc Natl Acad Sci USA 2004;101:3065–3070.
  12. Peden A, McCardle L, Head MW, Love S, Ward HJ, Cousens SN, Keeling DM, Millar CM, Hill FG, Ironside JW: Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia. Haemophilia 2010;16:296–304.
  13. Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW: Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004;364:527–529.
  14. Health Protection Agency: Fourth case of transfusion-associated variant-CJD. Health Protection Report 2007;1(3).
  15. Wroe SJ, Pal S, Siddique D, Hyare H, MacFarlane R, Joiner S, Linehan JM, Brandner S, Wadsworth JD, Hewitt P, Collinge J: Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report. Lancet 2006;368:2061–2067.
  16. Llewelyn CA, Hewitt PE, Knight RS, Amar K, Cousens S, Mackenzie J, Will RG: Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion. Lancet 2004;363:417–421.
  17. Ghani AC, Ferguson NM, Donnelly CA, Anderson RM: Predicted vCJD mortality in Great Britain. Nature 2000;406:583–584.
  18. Cousens SN, Vynnycky E, Zeidler M, Will RG, Smith PG: Predicting the CJD epidemic in humans. Nature 1997;385:197–198.
  19. Will RG, Alperovitch A, Poser S, Pocchiari M, Hofman A, Mitrova E, de Silva R, D’Alessandro M, Delasnerie-Laupretre N, Zerr I, van Duijn C: Descriptive epidemiology of Creutzfeldt-Jakob disease in six European countries, 1993–1995. EU Collaborative Study Group for CJD. Ann Neurol 1998;43:763–767.
  20. Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto A, Heinemann U, Breithaupt M, Varges D, Meissner B, Ladogana A, Schuur M, Haik S, Collins SJ, Jansen GH, Stokin GB, Pimentel J, Hewer E, Collie D, Smith P, Roberts H, Brandel JP, van Duijn C, Pocchiari M, Begue C, Cras P, Will RG, Sanchez-Juan P: Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain 2009;132:2659–2668.
  21. Zeidler M, Gibbs CJ Jr, Meslin F: WHO Manual for Strengthening Diagnosis and Surveillance of Creutzfeldt-Jakob Disease. Geneva, World Health Organization, 1998, pp 1–75.
  22. Prusiner SB: The prion diseases (review). Brain Pathol 1998;8:499–513.

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