COVID‐19‐associated immune‐mediated encephalitis mimicking acute‐onset Creutzfeldt‐Jakob disease

Beretta, Simone; Stabile, Andrea; Balducci, Claudia; DiFrancesco, Jacopo C.; Patruno, A; Rona, Roberto; Bombino, Michela; Capraro, Cristina; Andreetta, Francesca; Cavalcante, Paola; Moda, Fabio; Citerio, Giuseppe; Bogliun, G.; Ferrarese, Carlo

doi:10.1002/acn3.51479

Cited by 10 publications

(15 citation statements)

References 17 publications

(24 reference statements)

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“…These 61 articles comprised 47 on COVID-19-associated AIE, 1 case report on possible Bickerstaff encephalitis, 1 case series on both of the above subgroups, and 12 on vaccine-associated AIE. The articles included 52 case reports 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65 66 and 9 case series. 5 6 67 68 69 70 71 72 73 Fig.…”

Section: Resultsmentioning

confidence: 99%

“…Our inclusion criteria identified 48 (67.6%) cases of possible seronegative AIE in association with COVID-19. 6 15 16 21 22 29 31 36 38 40 41 42 43 44 53 54 55 56 57 59 60 61 62 63 67 68 70 73 Males were more commonly affected than females (sex ratio: 1.67). The median age was 60 years (IQR=46–66 years), which was higher than that in the definite-AIE subgroup.…”

Section: Resultsmentioning

confidence: 99%

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AI-CoV Study: Autoimmune Encephalitis Associated With COVID-19 and Its Vaccines—A Systematic Review

et al. 2022

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Background and Purpose Autoimmune encephalitis (AIE) following coronavirus disease 2019 (COVID-19) is an underexplored condition. This study aims to systematically review the clinico-investigational and pathophysiologic aspects of COVID-19 and its vaccines in association with AIE, and identify the factors predicting neurological severity and outcomes. Methods Relevant data sources were searched using appropriate search terms on January 15, 2022. Studies meeting the criteria for AIE having a temporal association with COVID-19 or its vaccines were included. Results Out of 1,894 citations, we included 61 articles comprising 88 cases: 71 of COVID-19-associated AIE, 3 of possible Bickerstaff encephalitis, and 14 of vaccine-associated AIE.There were 23 definite and 48 possible seronegative AIE cases. Anti-NMDAR (N-methyl-D-aspartate receptor; n =12, 16.9%) was the most common definite AIE. Males were more commonly affected (sex ratio=1.63) in the AIE subgroup. The neurological symptoms included alteredmental state ( n =53, 74.6%), movement disorders ( n =28, 39.4%), seizures ( n =24, 33.8%), behavioural ( n =25, 35.2%), and speech disturbances ( n =17, 23.9%). The median latency to AIE diagnosis was 14 days (interquartile range=4–22 days). Female sex and ICU admission had higherrisks of sequelae, with odds ratio (OR) of 2.925 (95% confidence interval [CI]=1.005–8.516)and 3.515 (95% CI=1.160–10.650), respectively. Good immunotherapy response was seen in42/48 (87.5%) and 13/13 (100%) of COVID-19-associated and vaccine-associated AIE patients, respectively. Sequelae were reported in 22/60 (36.7%) COVID-19 associated and 10/13 (76.9%) vaccine-associated cases. Conclusions The study has revealed diagnostic, therapeutic, and pathophysiological aspects of AIE associated with COVID-19 and its vaccines, and its differences from postinfectious AIE. Systematic review registration PROSPERO registration number CRD42021299215

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

AI-CoV Study: Autoimmune Encephalitis Associated With COVID-19 and Its Vaccines—A Systematic Review

et al. 2022

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“…There is also the evolving realization that SARS-CoV-2 accelerates and/or intensifies the pathomechanism of PrD and other types of progressive pro-inflammatory neurodegeneration, including AD. This is coupled with the growing recognition of significant cytokine-facilitated immune and pro-inflammatory responses to systemic SARS-CoV-2 invasion and a general viral-mediated hastening of the neurodegenerative disease process [ 53 , 54 , 59 , 63 , 72 , 73 , 74 ]. Cytokine-directed therapies may have some benefit in the clinical management of either COVID-19 or PrD [ 59 , 63 ].…”

Section: Discussionmentioning

confidence: 99%

“…Because of their unusual and atypical infective nature, PrDs and prion neurobiology have been intensively studied in considerable detail [ 19 , 20 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 ]. The brain- and CNS-abundant cellular prion sialoglycoprotein PrP c monomer consists of a constitutively expressed ~209-amino-acid, ~200 kDa glycosylated polypeptide containing a predominant internal α-helical region.…”

Section: Prion Disease (Prd) and Prion Neurobiologymentioning

confidence: 99%

“…Typically, activated microglia accumulate around PrP sc aggregates and release cytokines such as IL-1β that play important roles in the inflammatory pathogenesis of PrD and the cytokine storm syndrome [ 19 , 20 , 47 , 51 , 59 ]. PrDs such as CJD appear to be initiated and driven by the accumulation of abnormally folded, protease-resistant isoforms of PrP sc , which leads to neuropathologic spongiform changes that coincide with neuroinflammation, microglial activation, and an irreversible and fatal pro-inflammatory neurodegeneration [ 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 ].…”

Section: Prion Disease (Prd) and Prion Neurobiologymentioning

confidence: 99%

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SARS-CoV-2 Invasion and Pathological Links to Prion Disease

et al. 2022

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Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the causative agent of the COVID-19 disease, is a highly infectious and transmissible viral pathogen that continues to impact human health globally. Nearly ~600 million people have been infected with SARS-CoV-2, and about half exhibit some degree of continuing health complication, generically referred to as long COVID. Lingering and often serious neurological problems for patients in the post-COVID-19 recovery period include brain fog, behavioral changes, confusion, delirium, deficits in intellect, cognition and memory issues, loss of balance and coordination, problems with vision, visual processing and hallucinations, encephalopathy, encephalitis, neurovascular or cerebrovascular insufficiency, and/or impaired consciousness. Depending upon the patient’s age at the onset of COVID-19 and other factors, up to ~35% of all elderly COVID-19 patients develop a mild-to-severe encephalopathy due to complications arising from a SARS-CoV-2-induced cytokine storm and a surge in cytokine-mediated pro-inflammatory and immune signaling. In fact, this cytokine storm syndrome: (i) appears to predispose aged COVID-19 patients to the development of other neurological complications, especially those who have experienced a more serious grade of COVID-19 infection; (ii) lies along highly interactive and pathological pathways involving SARS-CoV-2 infection that promotes the parallel development and/or intensification of progressive and often lethal neurological conditions, and (iii) is strongly associated with the symptomology, onset, and development of human prion disease (PrD) and other insidious and incurable neurological syndromes. This commentary paper will evaluate some recent peer-reviewed studies in this intriguing area of human SARS-CoV-2-associated neuropathology and will assess how chronic, viral-mediated changes to the brain and CNS contribute to cognitive decline in PrD and other progressive, age-related neurodegenerative disorders.

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