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Epidemiology of CREUTZFELTD-JAKOB disease in PIEDMONT,1999-2000 : clinical and molecular analysis

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).

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Epidemiology of CREUTZFELTD-JAKOB disease in PIEDMONT,1999-2000 : clinical and molecular analysis

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  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 1999-2000Number of cases per year

  4. Incidence by age-groups

  5. Patients’ residence • Torino 17 • Cuneo 1 • Novara 1 • Vercelli 1 • Asti 1 • Alessandria - • Biella - • Aosta -

  6. Sporadic Creutzfeldt-Jakob disease in Piedmont 1999-2000 ( Piedmont and Valle d’Aosta :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 1999-2000Clinical 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 1999-2000Clinical 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 1999-2000PrPres typing and PRNP genotyping

  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.

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