Annals of Clinical Case Reports

Case Report
Published: 15 Apr, 2023

A Case Report of Creutzfeldt-Jakob Disease after mRNA
COVID-19 Vaccine
Karabudak S1*, Kurtoğlu AV1, Uslu F1 and Gürsoy AE2
1

Department of Neurology, Bezmialem Vakif University Hospital, Turkey

2

Department of Neurology, Basaksehir Cam and Sakura City Hospital, Turkey

Abstract
We report a rare case of Creutzfeldt-Jakob disease whose complaints started after a few weeks’mRNA COVID-19 vaccine. Prion diseases are neurodegenerative diseases which have longincubation periods. After clinical symptoms appear, it progresses rapidly to death. Beside clinicalsymptoms, EEG, Brain MRI and Protein 14-3-3 pozitivity support to diagnose. There is no effectivetreatment. In this paper, we present a case admitted to our clinic with rapidly progressive cognitive
impairment after mRNA COVID-19 vaccine along with protein 14.3.3 positivity.
Keywords: Creutzfeldt-Jakob disease; COVID-19; Vaccine; Immunity

Introduction

OPEN ACCESS*Correspondence:
Saniye Karabudak, Department ofNeurology, Bezmialem Vakif University
Hospital, Topkapı mah Vatan cad34225, Fatih-Istanbul, Turkey, Tel:+905387766357; Fax: +902124531883;
E-mail: saniyekarabudak@gmail.com
Received Date: 28 Mar 2023
Accepted Date: 10 Apr 2023
Published Date: 15 Apr 2023
Citation:
Karabudak S, Kurtoğlu AV, Uslu
F, Gürsoy AE. A Case Report of
Creutzfeldt-Jakob Disease after mRNA
COVID-19 Vaccine. Ann Clin Case Rep.
2023; 8: 2408.
ISSN: 2474-1655.
Copyright © 2023 Karabudak S. This is
an open access article distributed under
the Creative Commons Attribution
License, which permits unrestricteduse, distribution, and reproduction inany medium, provided the original work
is properly cited.

Many neurological diseases have been reported after COVID-19 infection [1,2]. Neurologicalmanifestations may be associated such as direct effects of the virus on the nervous system, parapost-infectious immune mediated disease, and neurologic complications of COVID-19 [2]. One of
the rare neurologic manifestations after covid infection is Creutzfeldt-Jakob disease [3]. Although
it is seen after covid infection, few cases have been reported due to covid vaccine in literature [4,5].
Prion diseases are characterized by a long incubation period and rapidly progressive, fatal clinic [6].
It is one of the main reasons of rapidly progressive cognitive disorders [7]. In this report; we aimed
to discuss the diagnosis and treatment in the light of the literature by sharing a case admitted to ourclinic with rapidly progressive cognitive impairment and general condition disorder after mRNA
COVID-19 vaccine along with protein 14.3.3 positivity.

Case Presentation
A 68-year-old male patient was admitted to our clinic with increasing speech disorder, confusion
and agitation for two days, intermittent tonic contraction in the left arm and leg, and pedaling
movements at night. He had the mRNA Covid vaccine 3 to 4 months ago, after 1 to 2 weeks simple
forgetfulness that would not affect daily living activities started. His speech disorder in the formof not being able to find words was added 2 months ago, in his background, he had hypertension,
mitral valve replacement, and Atrial Fibrillation (AF). He was using Warfarin sodium. In hisNeurological Examination (NM), he was apathetic, there was no spontaneous word output, and herarely took single orders. Left nasolabial sulcus was slightly indistinct-sol. Medical Research Council(MRC) grades were 4/5 on bilateral upper and lower limbs. No acute pathology was observed in
the brain tomography of the patient, who could not undergo MRI because the metal valve was notcompatible with MRI.
Due to the onset of symptoms after vaccination, he was hospitalized in the neurology service
with the preliminary diagnoses of infectious, autoimmune, paraneoplastic encephalitis, CreutzfeldtJakob disease, and to investigate the etiology of rapidly progressive cognitive impairment. In theclinical follow-up, the patient had an episodic and stereotypical pattern of focal seizures whichstarted absence for a few seconds by followed spasms in the left arm and then agitation. Considering
focal seizures. levetiracetam was added to his treatment, and 3 × 1000 mg/day was administeredgradually. Although his seizures decreased, lacosamide 50 mg 2 × 1/day was added. After that, nosimilar attacks were observed. EEG examinations performed 5 times in total during 2 weeks. They
showed diffuse disorganization in the patient. In malignancy screening examinations to investigate
paraneoplastic processes it was discovered mediastinal lymph nodes on thorax CT, but malignancy
was not considered. There was no FDG uptake in favor of malignancy in the whole-body PET
examination. Brain PET showed hypometabolism in the right parietofrontal cortex and superior

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Annals of Clinical Case Reports - Neurology

Karabudak S, et al.,Figure 1: Periodic wave activity showing asymmetry.

temporal lobe, which could be interpreted in favor of cerebrovasculardisease or subacute autoimmune encephalitic processes. Lumbar
puncture was made for investigating the etiology Cerebrospinal Fluid(CSF) protein: 35 mg/dL (N: 15-45 mg/dL), 3 leukocytes were seen.
Oligoclonal band, autoimmune encephalitis panel viral panel, atypicalcell, 14.3.3 protein, paraneoplastic antibodies were sent in CSF andserum. With the preliminary diagnosis of autoimmune encephalitis, 1
g/day Intravenous Methylprednisolone (IVMP) was given for 2 days.

proteins [8-10]. They made a basic manual reading of the vaccine
mRNA sequence. They discovered a total of sixteen UG tandem
repeats (ΨGΨG) besides the UG (ΨG) rich sequences in the vaccine
nucleic acid sequence. Two GGΨA sequences were also found. It is
unclear whether these mRNA sequence differences or the resulting
amino acid sequence differences lead to different levels of risk fordeveloping prion disease. Considering our patient and other patients
presented in the literature, it should be kept in mind and questionedthat symptoms may occur with vaccination in sporadic CJD cases.

Upon the development of CRP elevation on the second day,aspiration pneumonia was observed in the thorax CT. IVMP wasdiscontinued and anti-biotherapy was started.

References
1. Young MJ, O’Hare M, Matiello M, Schmahmann JD. CreutzfeldtJakob disease in a man with COVID-19: SARS-CoV-2-accelerated
neurodegeneration? Brain Behav Immun. 2020;89:601-3.

The patient whose steroid treatment could not be completed
was given 125 g Intravenous Immunoglobulin (IVIG) at 2 g/kg. The
patient, who had partial orientation to sound in the NM follow-ups,
but could not fully recover, was transferred to the Intensive Care
Unit (ICU) due to rapid ventricular response AF and deteriorationin general condition. Autoimmune encephalitis and paraneoplastic
antibodies resulted in negative when he was in ICU. Protein 14.3.3
was positive. Very low amplitude suspicious sharp wave paroxysms
were observed in the EEG examination taken at the 4th month of his
ICU follow-up (Figure 1).

2. Ellul MA, Benjamin L, Singh B, Lant S, Michael BD, Easton A, et al.
Neurological associations of COVID-19. Lancet Neurol [Internet].
2020;19(9):767-83.
3. Bernardini A, Gigli GL, Janes F, Pellitteri G, Ciardi C, Fabris M, et al.
Creutzfeldt-Jakob disease after COVID-19: Infection-induced prion
protein misfolding? A case report. Prion. 2022;16(1):78-83.
4. Perez JC, Moret-Chalmin C, Montagnier L. Towards the emergence of a
new form of the neurodegenerative Creutzfeldt-Jakob disease: Twenty-six
cases of CJD declared a few days after a COVID-19 vaccine; Jab. 2022.

The patient, whose clinical course progressed, died in the 5thmonth of her complaints and in the 2nd month of clinical worsening.

5. Simay S, Sungurtekin H. Creutzfeldt-Jakob disease after the coronavirus
disease-2019 vaccination. Turk J Intensive Care. 2022;20:61-4.

Discussion

6. Belay ED. Transmissible spongiform encephalopathies in humans. Annu
Rev Microbiol. 1999;53:283-314.

Prion diseases should be considered in patients applied clinic ofrapidly progressive cognitive impairment. The recent increase in thefrequency of diagnosis is remarkable. In the literature, more than one
case with CJD diagnosis and findings after covid vaccination has beenreported [4,5]. Discussions on the subject are mostly based on the factthat the mRNA in the mRNA COVID-19 vaccine has the potential
to bind to specific proteins and cause pathological misfolding [810]. Classen et al. analyzed the mRNA of mRNA COVID-19 vaccine
for its potential affinity to TAR DNA binding protein (TDP-43)
and Fused in Sarcoma (FUS) which are intracellular RNA binding
proteins [8-10]. They found that the mRNA of COVID-19 vaccine
has sequences that could convert these proteins into pathologic prion

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7. Geschwind MD. Rapidly progressive dementia. Continuum (MinneapMinn). 2016;22(2 Dementia):510-37.
8. Idrees D, Kumar V. SARS-CoV-2 spike protein interactions withamyloidogenic proteins: Potential clues to neurodegeneration. Biochem
Biophys Res Commun. 2021;554:94-98.
9. Classen JB. COVID-19 RNA based vaccines and the risk of prion disease.
Microbiol Infect Dis. 2021;5.1:1-3.
10. Classen JB. Review of COVID-19 vaccines and the risk of chronic
adverse events including neurological degeneration. J Med Clin Res Rev.
2021;5(3):1-7.

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