Confronting the Unexpected: A Case Report of Creutzfeldt - Jakob Disease in the Context of COVID-19
Received Date: August 19, 2023 Accepted Date: September 19, 2023 Published Date: September 22, 2023
doi: 10.17303/croa.2023.8.202
Citation: Yong II Shin (2023) Confronting the Unexpected: A Case Report of Creutzfeldt - Jakob Disease in the Context of COVID-19. Case Reports: Open Access 8: 1-6
Abstract
Creutzfeldt-Jakob disease (CJD) is a rare, but fatal neurodegenerative disease characterized by rapidly progressive neurological deficits including cognitive impairment, personality changes, aphasia, dysphagia, ataxia, and pyramidal and/or extrapyramidal symptoms [1,2]. CJD should be suspected in patients with rapidly progressive dementia syndrome.
Since the Coronavirus disease 2019 (COVID-19) pandemic, diverse neurological complications have been observed such as stroke, encephalitis, and Guillain-Barré syndrome [3]. Moreover, there have been case reports about temporary or longterm neurological sequelae after COVID-19 vaccines, but there is no strong evidence to support these findings [4].
This is a case of rapid neurological decline secondary to sporadic CJD that occurred several weeks post both asymptomatic COVID-19 infection and recent COVID-19 mRNA booster vaccination.
Keywords: Creutzfeldt-Jakob; COVID-19; Encephalitis; Encephalopathy; Neurodegeneration
Case Presentation
A 74-year-old female presented to the emergency room for evaluation of worsening aphasia, dysphagia, and ataxia for one week.
Three weeks prior to presentation, she was admitted to the hospital for confusion. She was diagnosed with focal seizures and discharged on Levetiracetam 500 milligrams twice a day. During the same admission, she tested positive for COVID-19 via polymerase chain reaction test. She was asymptomatic and had an unremarkable chest xray; therefore no treatment was indicated. Two months prior to admission, she was vaccinated with the second dose of COVID-19 mRNA, and did not experience any side effects. Her chronic medical problems included prediabetes and dyslipidemia.
On presentation, her physical exam was remarkable for expressive and receptive aphasia as well as right arm and leg drift. Complete blood count and comprehensive metabolic panel were within normal limits. Computerized tomography (CT) of the head showed patchy hypodensities possibly related to mild microvascular ischemic disease, but no other acute changes were seen. Magnetic resonance imaging (MRI) of the brain revealed restricted diffusion located in the lateral and paramedian aspects of the left parietal lobe, left frontal lobe, left insular cortex and basal ganglia (figure 1). A lumbar puncture was performed due to concern for encephalitis. Cerebrospinal fluid (CSF) analysis was significant for an elevated protein level of 79 mg/dL. Infectious work-up was negative (table 1). Electroencephalograms (EEG) showed background slowing indicative of general cerebral dysfunction.
After bacterial and viral encephalitis were ruled out, the patient received intravenous immunoglobulins (IVIG) for possible autoimmune encephalitis. Due to lack of clinical response, COVID-19 encephalitis was suspected. Therefore, 1 gram of intravenous methylprednisolone was administered for 5 days. Unfortunately, her neurological condition continued to rapidly decline and she became comatose on day 18 of her hospitalization. Ultimately, her 14-3-3 CSF assay and RT-QuIC returned positive. A diagnosis of probable sporadic CJD was made based on the Centers for Disease Control and Prevention (CDC) criteria (table 2).
The patient’s family declined a biopsy to confirm the diagnosis. After family discussion, the patient was ultimately transitioned to comfort care.
Discussion
An early diagnosis of a rare disease is challenging even in the best conditions, but it amplifies during a pandemic when isolation and social distancing is mandatory [5]. The described patient developed a rapid cognitive decline along with pyramidal symptoms which are commonly seen in CJD [6]. However, her clinical decline was initially attributed to COVID-19 encephalitis rather than CJD due to her recent SARS-CoV-2 infection [7].
A considerable amount of literature has been published about different neurological manifestations associated with SARS-CoV-2. A few authors hypothesized that COVID-19 can be a possible cause of sporadic CJD or that it can accelerate the course of the underlying neurodegenerative disease [3,8]. The hypothesis is that SARS-CoV-2 can potentially induce a neuroinflammatory state that leads to misfolding and aggregation of PrP. In fact, infectious agents have been reported as a possible risk factor for neurodegen erative diseases, causing protein misfolding and aggregation in selected brain regions [9]. There is also data suggesting that
SARS-CoV-2 binds to heparin binding proteins and accelerates the aggregation of pathological amyloid proteins present in the brain [10].
Notably, the above patient also received the COVID-19 mRNA vaccine one month prior to her first neurologic symptom. A few case reports describe a potential link between CJD and COVID-19 mRNA vaccine [4,11]. This may be due to vaccine contents binding to specific proteins causing pathologic misfolding. This relationship may be coincidental, and further studies are needed in order to make a definitive conclusion.
Acknowledgements
None.
Declaration of Interest Statement
The authors have no conflict of interest.
- de Silva R, Findlay C, Awad I, Harries-Jones R, Knight R, Will R (1997) Creutzfeldt-Jakob disease in the elderly. Postgrad Med J 73: 557-9.
- Bernardini A, Gigli GL, Janes F, Pellitteri G, Ciardi C et al. (2022) Creutzfeldt-Jakob disease after COVID-19: infection-induced prion protein misfolding? A case report. Prion 16: 78-83.
- Sisniega DC, Reynolds AS (2021) Severe Neurologic Complications of SARS-CoV-2. Curr Treat Options Neurol 23: 14.
- Anil Kuvandik, Ecenur Ozcan, Simay Serin, Hülya Sungurtekin (2022) Creutzfeldt-Jakob Disease After the COVID-19 Vaccination. Turk J Intensive Care.
- McMurran CE, Chaggar GH, Ugoya SO (2020) A patient with sporadic Creutzfeldt-Jakob disease: challenges of rare diseases in the COVID-19 era. Oxf Med Case Reports 2020: 113.
- Salehi P, Clark M, Pinzon J, Patil A (2022) Sporadic Creutzfeldt-Jakob disease. Am J Emerg Med 52: 1-3.
- Farhadian S, Glick LR, Vogels CBF, Thomas J, Chiarella J et al. (2020) Acute encephalopathy with elevated CSF inflammatory markers as the initial presentation of COVID-19. BMC Neurol 20: 248.
- Tayyebi G, Malakouti SK, Shariati B, Kamalzadeh L (2022) COVID-19-associated encephalitis or Creutzfeldt-- Jakob disease: a case report. Neurodegener Dis Manag 12: 29-34.
- De Chiara G, Marcocci ME, Sgarbanti R, Civitelli L, Ripoli C et al. (2012) Infectious agents and neurodegeneration. Mol Neurobiol 46: 614-38.
- Idrees D, Kumar V (2021) SARS-CoV-2 spike protein interactions with amyloidogenic proteins: Potential clues to neurodegeneration. Biochem Biophys Res Commun 554: 94-8.
- Folds Andrea J, Ullrich, Melanie-Belle, Htoo Sann, Chukus Anjeza (2022) "Sporadic Creutzfeldt-Jakob Disease After Receiving the Second Dose of Pfizer-BioNTech COVID-19 Vaccine" (2022). Internal Medicine 420.
FIGURE 1
Figure 1: Brain MRI (diffusion-weighted imaging) showing restricted diffusion predominantly in the lateral and paramedian aspects of the left parietal lobe,the paramedian left frontal lobe, left insular cortex and basal ganglia bilaterally
Tables at a glance
Figures at a glance