Deadly brain disease claims US man’s life, linked to Covid

A deadly brain disease that claimed the life of a 62-year-old man in the US has been linked to Covid-19, according to a case report.

The man was admitted to Mount Sinai Queens Hospital Center, with rapidly progressive dementia along with difficulty walking and myoclonus, revealed a paper published in the American Journal of Case Reports.

Two months prior to presenting to the hospital, he had developed diffuse bradykinesia, drooling, and dementia. He was noted to have difficulty walking.

He was still highly functioning in terms of work until he was found at home on the floor after a fall, unable to walk and confabulating, with intermittent brief rapid involuntary jerks involving 1 limb at a time, suggestive of myoclonic jerks.

On admission, he was found to be Covid-19 positive, but was afebrile and asymptomatic aside from the typical Covid respiratory manifestations.

The doctors established a “diagnosis of probable Prion disease (PrD) based on the positive result of protein detection in Cerebral spinal fluid along with a clinical picture of rapidly progressive dementia with bradykinesia and myoclonus,” said corresponding author Merjona Saliaj from the Department of Internal Medicine, at Queens Hospital.

The patient continued to deteriorate and died due to the rapid progression of his condition, Saliaj reported in the paper.

“Our case demonstrates the potential correlation of Covid with neurodegenerative conditions, especially prion disorders. While such cases are highly likely to be due to Covid-19, there is no definite evidence beyond coincidental findings. Future studies might be required to establish this correlation,” Saliaj said.

PrD is one of the rapidly progressive dementias and typically requires several diagnostic criteria to fulfil a probable diagnosis, as definite diagnosis is based on isolated brain biopsy. There has been much debate on a possible infectious aetiology of PrD.

Viral infections are commonly pathologic in most neurodegenerative conditions. In PrD, misfolded proteins can be contagious and act as infective proteins, regardless of the pathologic agent.

There is evidence that Covid-19 can result in neurologic manifestations, and neurodegeneration has been reported in the literature. There are several case reports describing parkinsonism after Covid-19, with Parkinson’s disease in particular noted in Covid-19.

“We were able to identify 4 cases in the literature comparable to our case with prion disorder happening after Covid-19 infection. While 2 cases had neurologic manifestations at onset, the 2 other cases had CJD-related symptoms at 2 weeks or 2 months after upper-respiratory symptoms,” Saliaj said.

Creutzfeldt-Jakob disease (CJD) due to prion disease is a fatal degenerative brain disorder. Early symptoms include memory problems, behavioural changes, poor coordination, and visual disturbances.

“Indeed, 2 cases had Covid-19-related manifestations, with 1 manifesting as fever and the other manifesting as fatigue, hyposmia, and hypogeusia. Counter-intuitively, 2 cases did not have any Covid-19-related manifestations prior to neurologic symptoms,” Saliaj said.

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