Barzilai University Medical Center

46 Research Day 2020 Barzilai University Medical Center 28 INCIDENCE OF CJD IN ISRAEL Walker M 1 , Nizan Z 1,2 , Appel S 1,2 , Kahana E 1 , Milo R 1,2 1 Department of Neurology, Barzilai University Medical Center, Ashkelon 2 Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva Background Creutzfeldt-Jakob disease (CJD) can present in 3 forms: sporadic (sCJD) with a uniform world incidence of ~1/10 6 , familial (fCJD) with mutations in the PrP protein, and infective (iCJD). fCJD accounts for <10% of all cases, however, in Israel where there is an unusual cluster of fCJD with the E200K mutation, most of the new cases are familial. Hypothesis As the number of E200K carriers increases overtime, there is a concern of iatrogenic transmission that may increase the incidence of sCJD in Israel. Objective Calculate the incidence of CJD in Israel over the years. Methods The Israeli CJD registry was screened for all patients with possible, probable and definite CJD (CDC diagnostic criteria) and for the various forms of CJD. Average annual incidence rate for each 3 years between 1963 and 2016 was calculated. Population at risk was derived from the Annual Publications of the Israeli Central Bureau of Statistics. Results There were 659 CJD patients with disease onset between 1963 and 2016. fCJD accounted for 428 (378 of Libyan origin, 45 of Turkish origin and 5 patients with the D178N mutation) and sCJD accounted for 231 (216 Jewish, 15 Arabs). The annual incidence for CJD increased from 0.52/10 6 in 1963-65 to 3.26/10 6 in 2014-16. The mean annual incidence of sCJD ranged from 0,79/10 6 to 1.14/10 6 in the years 1993-2016. Age adjustment eliminated the slight increase in incidence due to increased longevity. The mean annual incidence of fCJD in patients of Libyan origin increased from 86.8/10 6 in 1972 to 239.4/10 6 in 2014-16. Conclusion There is marked increase in the incidence of fCJD overtime. The stable incidence of sCJD overtime suggests no transmission of the disease. This research may serve as a basis for follow up and further evaluation of factors that may influence the incidence of CJD.