Monitoring Creutzfeldt-Jakob Disease (CJD) notification during Covid 19 Pandemic in ITALY (from 2017 to 2022)

Background: Creutzfeldt-Jakob disease (CJD) and related syndromes are rare human diseases belonging to the wide group of Transmissible spongiform encephalopathies (TSEs) or prion diseases. Humans TSEs include sporadic, genetic, iatrogenic and infectious forms . TSEs are caused by transmissible agents called prions wich replicates in the CNS leading to a characteristic neurodegenerative picture including spongiosis, glyosis, neuron loss and the deposition of the pathological prion protein (PrPTSE).Surveillance of human TSEs has been established in 1993 in Italy and contributed to the identification and recording of variant CJD in Europe and in Italy. Objectives: The objective of this retrospective study is to assess the incidence rate of CJD during 5 year period in Italy . We analyse data of CJD patients who were notified from 2017 to 2022 during, before and after covid pandemic, using data from the Italian Creutzfeldt-Jakob disease register. Methods Human TSE must be reported by law, and this must be done if there is the suspicion of TSE and if it is a definite confirmed case of TSE (Ministerial decree of December 21, 2001, published on G.U. n° 8 on January 10, 2002). The National Registry of CJD and correlated syndromes classifies each referred case based on established criteria from the European surveillance group. ,Based on clinical, test and laboratory information, each patient is classified as a: probable case of TSE;possible case of TSE;Non TSE-affected case. Based on genetic testing and medical history, the patient is then further classified as :Sporadic TSE case (sporadic CJD); Genetic TSE case (genetic CJD, GSS, FFI); CJD variant case; , sporadic fatal insomnia, Iatrogenic CJD case. data is inserted in an electronic database. This data is entered and updated by Registry personnel. Results Conclusion : This study contributes to our knowledge about CJD epidemiology during Covid -19 pandemic