Presentation on theme: "Epidemiology of CREUTZFELTD- JAKOB disease in PIEDMONT,1999-2000 : clinical and molecular analysis MariaTeresa Giordana, Marco Iudicello, Maria Luigia."— Presentation transcript:
Epidemiology of CREUTZFELTD- JAKOB disease in PIEDMONT, : clinical and molecular analysis MariaTeresa Giordana, Marco Iudicello, Maria Luigia Gianelli,Salvatore Monaco,GianLuigi Zanusso, Fabrizio Tagliavini, Roberto Testi, Adriano Chiò (Torino, Verona, Milano)
Sporadic Creutzfeldt-Jakob disease in Piedmont ( Piedmont and Valle dAosta :4,404,731 inhabitants) Incidence of sporadic CJD: 2.27/1.000,000/year (95% c.i., ) Annual death rate (2000): 1.6 / in Piedmont 1.2 / in Italy (I.S.S.)
Sporadic Creutzfeldt-Jakob disease in Piedmont Clinical features of 22 cases Age at onset : years Gender:14 women, 8 men Duration of symptoms: 2-20 months Typical EEG (PSCW): 12 cases protein in CSF: 22 cases
Sporadic Creutzfeldt-Jakob disease in Piedmont Clinical features Main symptom at onset : Cognitive,psychiatric 12 cases Ataxia, dyskinesias 10 cases Hyperintense basal ganglia in MRI: 8 cases
( Parchi P. 1995)
SPORADIC CJD in PIEDMONT PrPres typing and PRNP genotyping CODON 129MMMVVV? PrP type 1 (6 cases)411 PrP type 2 (5 cases)32 N.d. (3 cases)21
Comments The incidence rate, higher than those previously reported for Italy and other areas, most probably does not indicate an increase in sporadic CJD.
The apparent rise may depend on: Progressive aging of the population Improvement in case ascertainment Collection of cases by active surveillance Unification of autopsies in one center Excellent bio-molecular analysis
Creutzfeldt-Jakob disease in Piedmont – No case had the clinical and/or molecular features of the variantCJD. The present data are a good background for the future surveillance of CJD.