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21 September 2023: Articles

Prion Disease After COVID-19: A Case Report

Unknown etiology, Challenging differential diagnosis, Rare disease

Karim Makhoul A , Tova Beeber A , Ryan Cordero A , Areej Khan A , Merjona Saliaj A*

DOI: 10.12659/AJCR.940564

Am J Case Rep 2023; 24:e940564

Table 2. Post-COVID PrD case reports in the literature.

CaseAgeCOVID related manifestationsClinical manifestations related to CJDTime to onset of CJDTests supportive of CJD diagnosisProgressionDeath
Young 2020 []6 60Fever, PCR positiveConfusion, dementia, slowness, paucity of speech with paraphasic errors, unsteady gait with intermittent right-hand clenching, perseveration, anomia, impaired comprehension, and intermittent myoclonic jerks of the right armConfusion at onset with worsening over the next 2 weeksCSF RT-QuIC, 14-3-3 and T-tauBrain MRI diffusion restriction in the left hemisphere and left caudate and thalamusEEG 1 Hz periodic lateralized dischargesMutism, right hemiplegia, somnolence agitation2 months later
Beretta 2021 []4 64No fever or respiratory symptomsConfusion, disorientation, moderate aphasia, mild right hemiparesis, and irregular myoclonic jerks at the right limbs, with a Glasgow Coma Scale (GCS) 12 (eyes opening to verbal command, confused, localizing pain, not obeying commands)Symptoms at onset14-3-3 positiveRT-QuIC negativeEEG with left sided lateralized periodic dischargesMRI cortical ribbon of the left perisylvian regionsTotal recoveryNA
Bernardini 2022 []5 40March 2021 persistent fatigue and transient hyposmia and hypogeusiaSARS-CoV-2 serology showed positive anti-Receptor Binding Domain (RBD) Spike 1 total antibodies at high titerBlack shadows when closing his eyes, followed by dizziness, difficulty reading and worsening of balance. In June 2021 he noticed loss of coordination of the left arm2 monthsEEG recordings showed diffuse theta slowing with generalized triphasic 1–2 Hz periodic sharp-wave complexesvery high NfL levels. high total Tau levels, slightly reduced beta-amyloid 1–42 and normal phospho-Tau-181 and beta-amyloid 42/40 ratio. Real-Time Quaking-Induced Conversion (RT-QuIC) CSF testing for PrP was positivebedriddenWas alive at the time of publication
Olivo 2022 []7 COVID swab positiveinability to sign, compromised coordination and paresis to the right leg speech impairments, dystonia and myoclonus. mild right hemiparesis, non-fluent speech and bilateral myoclonus augmented by startleNeurologic manifestation at onsetMRI scan and highlighted the presence of a diffuse cortical ribboning in DWI periodic triphasic complexes were observed at EEG RT-QuIC results confirmed the probable diagnosis of CJDAkinetic mutismDeath

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923