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2733 A case of sporadic Creutzfeldt-Jakob Disease (sCJD) following viral vector COVID-19 vaccination
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  1. Mengchen Suo1,2,
  2. Neha Irani1,3,
  3. Wai Leong1,
  4. Jane Lock4 and
  5. Kevin O’Connor1,5,6
  1. 1Department of Neurology, Royal Perth Hospital, Perth, WA, Australia
  2. 2Department of Neurology, Sir Charles Gairdner Hospital, Nedlands, WA, Australia
  3. 3Department of Ophthalmology, Royal Perth Hospital, WA, Perth, WA, Australia
  4. 4Department of Ophthalmology, Sir Charles Gairdner Hospital, Nedlands, WA, Australia
  5. 5Department of Neurology, Joondalup Health Campus, Joondalup, WA, Australia
  6. 6Department of Immunology, Royal Perth Hospital, WA, Perth, WA, Australia

Abstract

A previously well and highly functioning 39-year-old male, had progressive short term memory loss and low mood, one month following first dose of AstraZeneca COVID-19 vaccination. He developed diplopia secondary to an acquired esotropia. Investigations were spuriously positive for anti-MUSK antibody and low positive VGKC antibody (negative CASPR2 and LGI1 antibody). Infective and autoimmune encephalitis screens were negative. MRI demonstrated diffuse cortical diffusion restriction with CSF negative for 14-3-3 and Rt-QuIC. Brain and whole body PET-CT showed global diffuse reduced activity, without occult malignancy. EEG showed nonspecific slowing. His condition deteriorated despite high dose steroids and IVIG.

The patient progressed to a rapidly progressive dementia with memory impairment, dysphasia, dysphagia, and myoclonus, 15 months following initial vaccination. Further treatment with plasmapheresis, IVIG and weekly IV methylprednisolone (Mayo Clinic autoimmune dementia protocol) did not improve his symptoms. Genetic testing showed homozygosity of methionine at PRNP codon 129 which confers a higher risk of sporadic CJD. Subsequent biopsy showed multifocal synaptophysin-like prion staining and focal perineuronal prion positive staining in keeping with the definitive diagnosis of Creutzfeldt-Jakob disease.

Conclusions We postulate that virus vector COVID vaccination may have accelerated or triggered sporadic CJD, in a genetically susceptible individual. Studies have highlighted a possible casual relationship between COVID-19 infection and CJD and other neurodegenerative disorders, due to a promotion of neuroinflammation and protein misfolding. Animal studies demonstrate accelerated transition from pre-clinical to clinical stages of prion disease with co-infection. This is to our knowledge the first case report of vaccine associated CJD.

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