NR AWHG
AU Imperiale,D.; Buffa,C.; Taraglio,S.; Bottacchi,E.; Testi,R.; Natale,G.; Chio,A.; Giordana,M.T.
TI Mortality from sporadic Creutzfeldt-Jakob disease in Piemonte, 1999-2005: clinical and molecular analysis
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-08
PT Konferenz-Poster
AB The average yearly mortality rate from sporadic Creutzfeldt-Jakob disease (CJD) in Italy, 1993-2000, is 1.04 cases per million inhabitants, while the reported rate in Piemonte, 1999-2000 is 1.41 per million (Puopolo et al 2003). Aim of the present investigation was to assess incidence in 1999-2005 and analyze trend in mortality rate, clinical, pathological, molecular data, and factors associated with survival duration of all incident cases of CJD in the resident population of Piemonte, 1999-2005. Since 2002 all incident cases are referred to the local Regional Human Prion Diseases Center established in Torino. Forty-eight (31 females,17 males) sporadic CJD cases and seven familial cases (E200K and L210 mutations) were identified. All sporadic cases were probable CJD. Autopsy was performed in 41 patients. Mean age at onset of symptoms was 66 year (range 36-82); mean disease duration 5.6 months (range 1-18). Average yearly mortality rate from sporadic CJD, 1999-2005 was 1.68, ranging from 0.91 in 2003 to 3.63 in 2005. Mortality rate by gender resulted higher for females (2.10) than males (1.22). Neuropathology confirmed the diagnosis in all cases; no case had the vCJD phenotype. MM at codon 129 was found in 74,4% of studied patients and was preferentially associated with PrPsc type 1; MV-type 2 was found in 10% of cases. Multivariate analysis of survival duration showed that diagnosis before 2001 and type 2 PrPres were significant predictors of longer survival (p 0,006 and 0,005 respectively). In conclusion, no significant variation in mortality rate was found in Piemonte, 1999-2005. The centralized referral of cases is of crucial importance in the continuing epidemiological surveillance of CJD. Supported by Regione Piemonte.
AD D. Imperiale, C. Buffa: Department of Neurology and Rehabilitation, Maria Vittoria Hospital, Torino, Italy; S. Taraglio, G. Natale: Department of Laboratory and Pathology, Maria Vittoria Hospital, Torino, Italy; E. Bottacchi: Department of Neurology, Hospital of Aosta, Aosta, Italy; R. Testi: Forensic Medicine, Amedeo di Savoia Hospital for Infectious Diseases,Torino, Italy; A. Chiò, M.T. Giordana: Department of Neuroscience, University of Torino, Italy. E-mail: daniele.imperiale@unito.it; mariateresa.giordana@unito.it
SP englisch
PO Italien