Rapidly evolving Creutzfeldt–Jakob disease in COVID-19: from early status epilepticus to fatal outcome, 2022, Olivo et al

[SNT Gatchaman]

Rapidly evolving Creutzfeldt–Jakob disease in COVID-19: from early status epilepticus to fatal outcome
Olivo, S., Furlanis, G., Buoite Stella, A. et al.

We report the case of a 70-year-old man coming to our attention for new onset refractory status epilepticus (NORSE) in a rapidly evolving CJD during SARS-CoV-2 co-infection. Our case report describes a fulminant CJD evolution associated with SARS-CoV-2 infection, which led to patient death after 15 days from admission. First EEG presented continuous diffuse spikes, sharp waves and sharp-and-slow wave complexes, pattern consistent with a non-convulsive status epilepticus (NORSE).

Our case supports how CJD with SARS-CoV-2 co-infection could be characterized by an accelerated evolution, as already hypothesize for others microorganism infections, and how the diagnosis might be more challenging due to its uncommon presentations, such as NORSE.

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[SNT Gatchaman]

Usually, the initial course of the disease is characterized by a subtle onset of psychiatric and neurological symptoms, with most of patients experiencing depression, anxiety, nervousness, autonomic disturbances, disruption of sleep–wakefulness rhythm, gait alterations, ataxia and myoclonus.

In the 40 days before the admission, the patient experienced non-specific ailment. The first complains were related to motor symptoms, including inability to sign, compromised coordination and paresis to the right leg. [...] neurological symptoms rapidly worsened, including speech impairments, dystonia and myoclonus.

Our case report describes a fulminant CJD associated with SARS-CoV-2 infection, which led to patient death after 15 days from the ED admission. To the best of our knowledge, only one study has previously reported a patient with rapidly progressive CJD with COVID-19 concomitant infection, suggesting a role of SARS-CoV-2 in spurring the progression of prion spreading.

Recent studies showed the role of SARS-CoV-2 in favoring the initiation of protein aggregation (including Aβ, α-synuclein, tau, prion, and TDP-43 RRM) leading to neurodegeneration and a link between neurodegeneration and cerebral inflammation.