Impact of the COVID-19 pandemic on Creutzfeldt-Jakob disease surveillance and patient care in the United Kingdom.

BACKGROUND AND PURPOSE: Creutzfeldt-Jakob disease (CJD) is lethal and transmissible. We assessed the impact of the COVID-19 pandemic on UK CJD surveillance. We hypothesized that (i) disruptions prolonged diagnostic latency; (ii) autopsy rates declined; and (iii) COVID-19 infection negatively affected diagnosis, care, and survival. METHODS: We retrospectively investigated the first year of the pandemic, using the preceding year as a comparator, quantifying numbers of individuals assessed by the UK National CJD Research & Surveillance Unit for suspected CJD, time to diagnosis, disease duration, and autopsy rates. We evaluated the impact of COVID-19 status on diagnosis, care, and survival in CJD. RESULTS: A total of 148 individuals were diagnosed with CJD in the pandemic (from a total of 166 individuals assessed) compared to 141 in the comparator (from 145 assessed). No differences were identified in disease duration or time to diagnosis. Autopsy rates were unchanged. Twenty individuals had COVID-19; 60% were symptomatic, and 10% had severe disease. Disruptions in diagnosis and care were frequently identified. Forty percent of COVID-19-positive individuals died; however, COVID-19 status did not significantly alter survival duration in CJD. CONCLUSIONS: The COVID-19 pandemic has not impacted UK CJD case ascertainment or survival, but diagnostic evaluation and clinical care of individuals have been affected.

Application of telehealth for comprehensive Creutzfeldt-Jakob disease surveillance in the United Kingdom.

Creutzfeldt-Jakob disease (CJD) is a fatal human prion disease. Surveillance systems operate globally with the goals of accurate in-life case ascertainment, appropriate public health interventions to minimise secondary transmission, and monitoring trends in disease epidemiology. The UK experienced the highest incidence of variant CJD (vCJD) in the world following widespread population exposure to bovine spongiform encephalopathy (BSE). 178 cases of vCJD have been identified in the UK by the National CJD Research & Surveillance Unit (NCJDRSU), including three cases of secondary transmission via blood transfusion. The NCJDRSU performs high-fidelity surveillance, assessing all cases of suspected CJD referred to the unit. COVID-19 has caused widespread disruption to healthcare and poses a threat to services. The NCJDRSU converted to telehealth-based surveillance in March 2020. We report the results of the application of telehealth for comprehensive CJD surveillance during the first four months of the pandemic. 59 cases were assessed for suspected CJD. In 52 cases the relatives were interviewed for an informant history, by video conference or telephone call. 34 patients underwent video examination; 1 case was examined in-person. MRI images were assessed in all cases and 46 underwent CSF testing. Feedback was obtained from interviewees and the NCJDRSU team on their experiences. 50 cases were diagnosed with sporadic CJD; 5 received an alternative diagnosis, and the remaining 4 remained unresolved, with further investigations underway. Telehealth significantly reduced time taken to assessment compared to in-person assessments in 2019. Telehealth is an effective way to provide comprehensive CJD surveillance at a national level.