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Background Variant Creutzfeldt–Jakob disease (vCJD), is a form of CJD associated with consumption of tissues from cattle infected with bovine spongiform encephalopathy. Confirmation requires cerebral histopathologic examination, ascertaining spongiform change and extensive prion protein deposition with florid plaques. In Greece, the disease has been included in the Mandatory Notification System since 2004 however no reference laboratory has been available since 2009 due to low global occurrence. In 2014, the surveillance system was strengthened to also include possible and probable cases based on the non-confirmatory diagnostic criteria of the EU case definition. Hereby we present the results of the surveillance for 2014–2018. Methods A new reporting form for CJD was introduced at the national level, including fields aligned with EU case definition criteria for vCJD (preconditions, clinical, diagnostic, and epidemiological). The non-confirmatory diagnostic criteria for the classification of probable and possible vCJD cases were based on examinations routinely available at hospitals, namely EEG and cerebral MRI. Reported CJD cases were assessed according to the fulfillment of required combinations of EU criteria for possible, probable, and confirmed vCJD cases. Results From 2014 to 2018, 37 CJD cases were reported, with a median age of 67 years (IQR: 58–73) concerning mostly females (71.3%, 95% CI: 56.7–85.9%). Among the reported cases, 6 (16.2%) fulfilled only the preconditions, 8 (21.6%) fulfilled the preconditions and the clinical criteria, 21 (56.8%) had a compatible EEG, and 7 (18.9%) had a compatible MRI brain scan. Confirmatory examination was not performed to any of the CJD cases; however, no case was classified as possible or probable vCJD based on the EU case definition. Conclusion Notification of CJD patients from 2014 to 2018 in Greece, yielded no vCJD cases. The possible re-emergence of vCJD renders continuous surveillance of the disease imperative. Current EU case definition can support the surveillance of vCJD, by screening CJD patients for possible and probable vCJD cases with the utilization of conventional diagnostic examinations, available in most hospitals. Disclosures All authors: No reported disclosures. |
