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Creutzfeldt-Jakob Disease After the COVID-19 Vaccination

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Reports of neurological problems are increasing for the clinical presentation of coronavirus disease-2019 (COVID-19). The clinical presentation reported in this study seemed to be a combination of nonspecific complications of the systemic disease, inflammation of the cerebrovascular system, and the effects of a direct viral infection. Creutzfeldt-Jakob disease, a spongiform encephalopathy caused by prions, is characterized by a severe neurological destruction, which has an extremely high mortality. In this publication, we presented a patient who was admitted to the Pamukkale University Anesthesiology Intensive Care Units with the neurological findings that developed after the COVID-19 vaccine (CoronaVac, Sinovac Life Sciences, Beijing, China). The patient died due to the progressive neurological disorders. In cases where rapidly progressive neurological disorders are observed, Creutzfeldt-Jakob disease should be considered and the role of immunity-related conditions in the progression of the disease should be investigated.
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CASE REPORT / OLGU SUNUMU
©Copyright 2022 by Turkish Society of Intensive Care
Turkish Journal of Intensive Care published by Galenos
Publishing House.
61
Turk J Intensive Care 2022;20:61-64
ÖZ
Koronavirüs hastalığı-2019 (COVID-19) hastalığının klinik seyrinde gelişen nörolojik problemlerle
ilgili raporlar artmaktadır. Bu klinik tablo, sistemik hastalığın spesifik olmayan komplikasyonları,
serebrovasküler sistem iltihabı veya doğrudan viral enfeksiyonun etkilerinin bir kombinasyonu gibi
görünmektedir. Prionların neden olduğu süngerimsi bir ensefalopati olan Creutzfeldt-Jakob hastalığı,
şiddetli nörolojik yıkım ile karakterizedir ve son derece yüksek bir ölüm oranına sahiptir. Bu yayında,
Pamukkale Üniversitesi Anesteziyoloji Yoğun Bakım Ünitesi’ne COVID-19 aşısı (CoronaVac, Sinovac
Life Sciences, Beijing, China) sonrası gelişen nörolojik bulgularla başvuran bir hastayı sunduk. Hasta
ilerleyici nörolojik bozukluklar nedeniyle öldü. Hızla ilerleyen nörolojik bozuklukların görüldüğü
durumlarda Creutzfeldt-Jakob hastalığı düşünülmeli ve bağışıklıkla ilgili durumların hastalığın
ilerlemesindeki rolü araştırılmalıdır.
Anahtar Kelimeler:
COVID-19, aşı, Creutzfeldt-Jakob hastalığı, prion proteini
ABSTRACT
Reports of neurological problems are increasing for the clinical presentation of
coronavirus disease-2019 (COVID-19). The clinical presentation reported in this study seemed
to be a combination of nonspecific complications of the systemic disease, inflammation of the
cerebrovascular system, and the effects of a direct viral infection. Creutzfeldt-Jakob disease, a
spongiform encephalopathy caused by prions, is characterized by a severe neurological destruction,
which has an extremely high mortality. In this publication, we presented a patient who was admitted
to the Pamukkale University Anesthesiology Intensive Care Units with the neurological findings
that developed after the COVID-19 vaccine (CoronaVac, Sinovac Life Sciences, Beijing, China).
The patient died due to the progressive neurological disorders. In cases where rapidly progressive
neurological disorders are observed, Creutzfeldt-Jakob disease should be considered and the role
of immunity-related conditions in the progression of the disease should be investigated.
Keywords:
COVID-19, vaccine, Creutzfeldt-Jakob disease, prion protein
E-mail : anilkuvandik@gmail.com
Phone : +90 544 636 99 32
ORCID ID : orcid.org/0000-0002-7555-6076
Anıl Kuvandık Ph.D. (),
Pamukkale University Faculty of Medicine,
Department of Anesthesiology and Reanimation,
Denizli, Turkey
Anıl Kuvandık, Ecenur Özcan, Simay Karaduman,
Hülya Sungurtekin
Pamukkale University Faculty of Medicine,
Department of Anesthesiology and Reanimation,
Denizli, Turkey
Received/Geliş Tarihi : 12.10 .2021
Accepted/Kabul Tarihi : 21 .12.202 1
Anıl Kuvandık,
Ecenur Özcan,
Simay Karaduman,
Hülya Sungurtekin
Koronavirüs Hastalığı-2019 Aşısı Sonrası
Creutzfeldt-Jakob Hastalığı
Creutzfeldt-Jakob Disease After the Coronavirus
Disease-2019 Vaccination
DOI: 10.4274/tybd.galenos.2021.91885
Introduction
Neurological effects from coronavirus disease-2019
(COVID-19) infections are now documented in scientic
studies (1). Neurological manifestations may be related such
as direct effects of the virus on the nervous system, para-
post-infectious immune mediated disease, and neurologic
complications of the systemic effects of COVID-19 (2).
Unvaccinated individuals are at higher risk of serious
illness from COVID-19 infection, which can cause temporary
or long-term neurological effects in some patients.
Autoimmunity and the opposing condition, metabolic
syndrome, are well known adverse events caused by
vaccines (3). COVID-19 infections are related to the
induction of autoantibodies and autoimmune disease which
makes it more than reasonable a vaccine can do the same.
There is no evidence that the COVID-19 vaccines lead to
neurodegenerative diseases as far as we know.
In this case report, a patient who was diagnosed with
Creutzfeldt-Jakob disease (CJD) after COVID-19 vaccine was
examined.
Case Report
A 82-year-old female patiet with a known diagnosis of
hypertension and dementia started to have tremors and
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Turk J Intensive Care 2022;20:61-64
Kuvandık et al. Prion Disease After COVID-19 Vaccination
weakness on the right side of her body. The patient’s
ndings emerged one day after the rst dose of COVID-19
CoronaVac vaccine was administered. She admitted to the
hospital with the addition of symptoms such as regression in
her state of consciousness, inability to recognize the people
around her, impaired vision, impaired place-time orientation
and meaningless shouts at the following month. She was
admitted to our neurology unit hospital with a diagnosis of
encephalitis and subdural hematoma.
On admission, she has myoclonic contractions
and disorientation in her right extremities. She has no
ndings in cranial nerve examinations. Clonus, rigidity and
hyperreexia were present in the right half of the body.
Glasgow coma scale (GCS) was 10/15 on neurological
examination. Laboratory studies such as liver and kidney
function tests, electrolytes, complete blood count, blood
gas analysis, coagulation tests, thyroid function tests,
autoimmune markers, viral encephalitis markers were done.
Cerebrospinal uid (CSF) samples were taken and examined.
Electroencephalograghy (EEG) examination was performed.
Blood, urine, respiratory secretions and CSF cultures were
made. No abnormal laboratory nding was found in blood
tests. Autoimmune encephalopathy panels were negative.
EEG showed paroxysmal mixed sharp and sharply slow
paroxysms, diffuse slowing of cerebral bioelectrical activity
without lateralization and localization. She received pulse
steroid and intravenous immunoglobulin G (IVIG) therapy
with a diagnosis of autoimmune encephalitis in the
neurology service. When GCS dropped to 4 in the neurology
service, she was intubated and hospitalized in our intensive
care unit. Brain and thorax computed tomography and
cranial magnetic resonance imaging (MRI) examinations
were performed. She received empirical antibiotics and
antiepileptic therapy for meningitis prophylaxis in addition
to pulse steroid and intravenous IVIG therapy in the
intensive care unit. Transesophageal echocardiography was
performed for infective endocarditis. Diffusion-weighted
MRI revealed cortical diffusion restriction in the left parietal,
occipital, temporal lobes and the right occipital lobe. In the
uid-attenuated inversion recovery (FLAIR) examination,
hyperintensity was observed in the same regions (Figure 1).
The patient was diagnosed with sporadic CJD after the
14-3-3 protein test was positive, MRI ndings, physical
examination, and evaluation of the clinical history. As a
result of her examinations, viral encephalitis, autoimmune
encephalitis and subdural hematoma were excluded. The
patient died as a result of the progressive course of the
disease. For the presentation of the patient, the patient was
allowed their relatives.
Figure 1. Brain magnetic resonance imaging (MRI) findings in this case. A,B,C,D: Diffusion-weighted MRI shows cortical diffusion restriction in the left
parietal, occipital, temporal lobes and the right occipital lobe. E,F,G,H: FLAIR examination shows hyperintensity in the same regions
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Turk J Intensive Care 2022;20:61-64
Kuvandık et al. Prion Disease After COVID-19 Vaccination
Discussion
COVID-19 is an unprecedented threat that is straining
health system capacities around the world. Neurological
symptoms develop in 17.3% to 36.4% of patients in the
acute phase of the disease, and 25% of these are caused
by central nervous system involvement. Central nervous
system involvement is mostly seen as viral meningitis or
encephalitis. The most common neurological symptoms are
headache, dizziness and changes in consciousness (4).
Possible mechanisms in the neurological involvement
of COVID-19; viral encephalitis, systemic inammation,
dysfunction of peripheral organs and cerebrovascular changes.
These mechanisms enhance neurological symptoms by
aggravating a pre-existing neurological disorder or initiating
a new disease (5). In COVID-19, there is an increase in
cytokines and inflammatory mediators resulting from
systemic inammation. Systemic inammation, on the other
hand, supports cognitive decline and neurodegenerative
diseases (6). Authors (7) found prion related sequences in
the COVID-19 spike protein which were not found in related
coronaviruses. It was also reported a case of prion disease,
CJD, initially occurring in a man with COVID-19 (1).
Vaccination is an effective strategy to reduce the burden
of preventable diseases. However, many clinical studies
have revealed that various vaccines may be associated with
different neurological disorders and autoimmune pathologies.
Although some studies show that neurological disorders that
develop after vaccination may not be related to the vaccine,
these results may be coincidental (8). In addition, reporting
of post-vaccine-related adverse events may increase the
hesitation of the public about vaccination and support
vaccination opposition. No article has been found in the
literature about the COVID-19 vaccines causing encephalitis.
CJD is a very rare, rapidly progressing, contagious
neurodegenerative disease with a mortality rate of 100%
caused by prion proteins. Although CJD is generally sporadic,
it can occur in 10% of cases with familial autosomal
dominant inheritance (9). The neuron losses in the gray
matter and the many vacuoles in the central nervous system
are responsible for the classic sponge-like appearance of the
brain and the emergence of specic clinical symptoms in
CJD. This infectious disease with fatal consequences is of
particular importance as it can be iatrogenically transmitted
to healthcare personnel and other patients (10).
Sporadic CJD usually begins with nonspecic symptoms
in older ages. Often there are personality disorders,
depression, sleep disorders and weight loss. Behavioral
problems and cognitive dysfunction are important
symptoms. Brain biopsy is the gold standard in diagnosis.
Since surgery is risky and samples cannot be taken from
the affected area all the time, brain biopsy can be performed
in uncertain diagnoses after all non-invasive diagnostic
methods are performed (11). Checking 14-3-3 protein and
tau protein in CSF is important in the diagnosis of sporadic
CJD. These proteins are markers of neuronal destruction
and their concentration increases in the later stages of the
disease. In the absence of clinical signs, the 14-3-3 protein
has no value and is not specic for CJD. Real-time quaking-
induced conversion (RT-QuIC) test is an examination that
detects the abnormal scrapie form of the prion protein
(PrPsc) and provides a denitive diagnosis (12). It was used
The Centers for Disease Control and Prevention’s criteria for
CJD diagnosis in our case (13). Our patient had neurological
symptoms and cognitive dysfunction. CSF examination was
positive for 14-3-3 protein. We did not have the opportunity
to do the RT-QuIC test.
Periodic sharp wave complexes (PSWC) in EEG are
found in 2/3 of patients with sporadic CJD and are among
the diagnostic criteria (13). The spikes on the EEG are
independent of typical signs of myoclonic seizures and
are associated with the fusion of dendritic membranes in
neurons. Although PSWCs are initially lateralized, they are
seen in bilateral frontal localization in progressive disease
(14). In our case, mixed and slow sharp waves observed
in EEG were not lateralized. In MRI, the high intensity
signal pattern in T2/FLAIR sequences is compatible with
astrogliosis and hyperintensity in diffusion-weighted imaging
with the formation of vacuoles and prion protein in the
brain. MRI images correlate with symptoms and clinical
ndings, but hyperintensity decreases in the later stages
of the disease and cortical atrophy may be the only nding.
Therefore, in the absence of specic imaging ndings, if CJD
is considered in the differential diagnosis, attention should
be paid to when the clinical ndings begin (15). In our case,
cortical diffusion restriction was detected in the left parietal,
occipital, temporal lobes, and right occipital lobe on MRI.
Hyperintensity was observed in the same regions in FLAIR
examination.
The onset of acute neurological symptoms after COVID-
19 vaccination suggested that there might be an adverse
effect related to the vaccine. Suppression of immunity after
vaccination may have accelerated the emergence of prion
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Turk J Intensive Care 2022;20:61-64
Kuvandık et al. Prion Disease After COVID-19 Vaccination
disease or the onset of the disease may be coincidental. In
order for CJD diagnosis to be associated with the vaccine,
the cause-effect relationship between them must be
revealed. Therefore, further studies are needed in this area.
Ethics
Informed Consent: For the presentation of the patient, the
patient was allowed their relatives.
Peer-review: Externally peer-reviewed.
Authorship Contributions
Surgical and Medical Practices: A.K., E.Ö., S.K., H.S.,
Concept: A.K., Design: A.K., Data Collection and/or
Processing: A.K., E.Ö., Analysis and/or Interpretation: A.K.,
E.Ö., S.K., H.S., Literature Search: A.K., E.Ö., Writing: A.K.,
E.Ö.
Conict of Interest: No conict of interest was declared by
the authors.
Financial Disclosure: The authors declared that this study
received no nancial support.
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Creutzfeldt-Jakob disease is a very rare, progressive neurodegenerative disorder that is incurable and always fatal. It is one of the transmissible spongiform encephalopathies caused by prions. Multiple vacuoles in neuropil and neuronal loss in the gray matter gives the classical sponge-like appearance of brain and are responsible for the typical clinical symptoms. In this report, we present 4 cases referred to the neurology department of Uludağ University with neurological symptoms. Patients were evaluated with electroencephalogram and magnetic resonance imaging, and performed brain biopsies for further investigation. For definitive diagnosis of Creutzfeldt-Jakob disease, accumulation of prion protein in brain was detected immunohistochemically. Patients died within weeks in consequence of rapid progression of the disease. Although Creutzfeldt-Jakob disease is an infrequent disorder, when a patient presents with characteristic clinical symptoms such as rapidly progressive dementia with myoclonus, the diagnosis of Creutzfeldt-Jakob disease should be taken into consideration.