Background

Creutzfeldt-Jakob disease (CJD) is lethal and transmissible. We assessed the impact of the COVID-19 pandemic on UK CJD surveillance. We hypothesised: 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 (NCJDRSU) 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

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. 20 individuals had COVID-19; 60% were symptomatic and 10% had severe disease. Disruptions to diagnosis and care were frequently identified. 40% 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.