1. Epidemiology of CREUTZFELTD- JAKOB disease in PIEDMONT,1999-2000 : clinical and molecular analysis MariaTeresa Giordana, Marco Iudicello, Maria Luigia Gianelli,Salvatore Monaco,GianLuigi Zanusso, Fabrizio Tagliavini, Roberto Testi, Adriano Chiò (Torino, Verona, Milano)

2. Creutzfeldt-Jakob disease in Piedmont – 1999-2000 Sporadic CJD : 22 cases Definite : 15 Probable : 7 Iatrogenic CJD : 0 Familial CJD : 3 (E200) Variant CJD : 0

3. Sporadic Creutzfeldt-Jakob disease in Piedmont 1999-2000 Number of cases per year By date of onset of disease Resident in Piedmont 199986 200013 By date of death 199986 200077

4. Incidence by age-groups

5. Patients residence Torino17 Cuneo1 Novara1 Vercelli1 Asti1 Alessandria- Biella- Aosta-

6. Sporadic Creutzfeldt-Jakob disease in Piedmont 1999-2000 ( Piedmont and Valle dAosta :4,404,731 inhabitants) Incidence of sporadic CJD: 2.27/1.000,000/year (95% c.i., 1.40-3.48) Annual death rate (2000): 1.6 /1.000.000 in Piedmont 1.2 /1.000.000 in Italy (I.S.S.)

7. Sporadic Creutzfeldt-Jakob disease in Piedmont 1999-2000 Clinical features of 22 cases Age at onset : 48- 77 years Gender:14 women, 8 men Duration of symptoms: 2-20 months Typical EEG (PSCW): 12 cases 14.3.3 protein in CSF: 22 cases

8. Sporadic Creutzfeldt-Jakob disease in Piedmont 1999-2000 Clinical features Main symptom at onset : Cognitive,psychiatric 12 cases Ataxia, dyskinesias 10 cases Hyperintense basal ganglia in MRI: 8 cases

9. ( Parchi P. 1995)

10. SPORADIC CJD in PIEDMONT 1999-2000 PrPres typing and PRNP genotyping CODON 129MMMVVV? PrP type 1 (6 cases)411 PrP type 2 (5 cases)32 N.d. (3 cases)21

11. Comments The incidence rate, higher than those previously reported for Italy and other areas, most probably does not indicate an increase in sporadic CJD.

12. 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

13. Creutzfeldt-Jakob disease in Piedmont – 1999-2000 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.