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Epidemiology of CREUTZFELTD- JAKOB disease in PIEDMONT,1999-2000 : clinical and molecular analysis MariaTeresa Giordana, Marco Iudicello, Maria Luigia.

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Presentation on theme: "Epidemiology of CREUTZFELTD- JAKOB disease in PIEDMONT,1999-2000 : clinical and molecular analysis MariaTeresa Giordana, Marco Iudicello, Maria Luigia."— Presentation transcript:

1 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)

2 Creutzfeldt-Jakob disease in Piedmont – 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 Number of cases per year By date of onset of disease Resident in Piedmont By date of death

4 Incidence by age-groups

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

6 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.)

7 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

8 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

9 ( Parchi P. 1995)

10 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

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 – 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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