NR AWHH
AU Imperiale,D.; Giordana,M.T.; Taraglio,S.; Bottacchi,E.; Atzori,C.; Filippi,P.; Cassano,D.; Testi,R.; Natale,G.; Buffa,C.
TI Epidemiology of human prion diseases in Piemonte and Valle d'Aosta in 2002-2005 period: a report of the first 4-year activity of the regional human prion disease center
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Poster sessions EPI-09
PT Konferenz-Poster
AB Since 2002, laboratory investigations and autopsies of suspected Creutzfeldt-Jakob disease (CJD) and the other prion diseases cases from Piemonte and Valle d'Aosta (North-West Italy) have been all unified in the Regional Human Prion Disease Center in "Maria Vittoria" and "Amedeo di Savoia" hospitals in Torino. These activities have paralleled the recording of patients' clinical and instrumental data to get a comprehensive view of human prion diseases in our districts. We aimed to analyse the epidemiology of human prion diseases in Piemonte and Valle d'Aosta in 2002-2005 years. In the whole period 2002-2005 a definite diagnosis of prion disease was made in 38 patients (24 females, 14 males). Sporadic CJD was diagnosed in 30 cases whereas sporadic fatal insomnia in 2 ones. PRNP gene sequencing disclosed a mutation in 6 patients (4 E200K, 1 P210L and 1 D178N). No case of accidentally transmitted or variant CJD was reported. The annual mortality rate for all prion diseases per million of inhabitants was 2.12 (CI 95% 0.79-3.45) in 2002, 0.83 (CI 95% 0.01-1.65) in 2003, 1.67 (CI 95% 0.50-2.84) in 2004 and 3.10 (CI 95% 1.51-4.69) in 2005. Mortality rates of prion diseases in Piemonte and Valle d'Aosta in 2002-2005 have paralleled the rates observed in the whole Italy and also the strikingly high value observed in 2005 is not significantly different from the national value. The unification of autopsies and specific laboratory investigations in one center may represent a good model to improve case ascertainment and active surveillance on human prion diseases. Regional Human Prion Disease Center is supported by the Regione Piemonte (Ricerca Sanitaria Finalizzata 2002-2003-2004)
AD D. Imperiale, M.T. Giordana, S. Taraglio, C. Atzori, P. Filippi, D. Cassano, R. Testi, G. Natale, C. Buffa: Centro Diagnosi Osservazione Malattie Prioniche D.O.M.P. - A.S.L. 3 Torino, Italy; E. Bottacchi: Divisione di Neurologia - Ospedale Regionale di Aosta, Address: Ospedale Maria Vittoria Via Cibrario 72, 10144 Torino, Italy. E-mail: imperiale@asl3.to.it
SP englisch
PO Italien