Creutzfeldt-Jakob disease in a man with COVID-19: SARS-CoV-2-accelerated neurodegeneration?

Abstract

We describe a man whose first manifestations of Creutzfeldt-Jakob disease occurred in tandem with symptomatic onset of coronavirus disease 2019 (COVID-19). Drawing from recent data on prion disease pathogenesis and immune responses to SARS-CoV-2, we hypothesize that the cascade of systemic inflammatory mediators in response to the virus accelerated the pathogenesis of our patient's prion disease. This hypothesis introduces the potential relationship between immune responses to the novel coronavirus and the hastening of preclinical or manifest neurodegenerative disorders. The global prevalence of both COVID-19 and neurodegenerative disorders adds urgency to the study of this potential relationship.

Keywords: COVID-19; Neurodegeneration; Prion disease; Psychoneuroimmunology.

Fig. 1

EEG recording demonstrates frequent 1 Hz left-sided lateralized periodic discharges, with diffuse delta-theta slowing of the background more prominently on the left.

Fig. 2

A – D: Brain MRI findings in this case. Diffusion weighted images from A, superior to D, inferior brain regions showing asymmetric restricted diffusion (bright signal, arrows) in the cortical ribbon more prominently in the left hemisphere in prefrontal, posterior parietal and retrosplenial cortices, as well as the insula and cingulate gyrus, with faint signal abnormality also in the body of the caudate nucleus (in C) and pulvinar of thalamus (in D). E-F: 18-FDG PET findings in this case, showing hypometabolism in areas of restricted diffusion.