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Proceedings SNO “Percorsi clinici in Neuroscienze”

A1 A2 A3 A4

B1 B2 B3 B4

Figure 1. Representative MRI images showing coronal DWI (A1; B1), ADC (A2; B2) and FLAIR (A3, A4; B3, B4) sequences of the
same slice and axial FLAIR sequence, performed in the subacute phase (day 10) (A) and post-acute phase (day 50) (B). Abnormal
cortical areas are indicated by arrows. Notably, ADC map does not show commensurate hypointensity in the anterior cingulate and
insula, where the DWI and FLAIR cortical hyperintensity was present.

DISCUSSION cephalitis, which could be explained by cytokines re-
lease within the CNS and related neuroinflammation.
We report for the first time a probable association be-
tween COVID-19 infection and a peculiar type of im-
mune-mediated encephalitis, which closely resem- REFERENCES
bles acute-onset sCJD.
Rapidly progressive cognitive dysfunction, focal my- 1. Hermann P, Laux M, Glatzel M, Matschke J, Knipper T,
Goebel S, Treig J, Schulz-Schaeffer W, Cramm M,
oclonus, EEG evolution to periodic sharp wave com-
Schmitz M, Zerr I. Validation and utilization of amended
plexes, bilateral cortical DWI hyperintensities on diagnostic criteria in Creutzfeldt-Jakob disease surveil-
brain MRI and 14.3.3 protein in the CSF were pres- lance. Neurology 2018; 91 (4): e331-e338.
ent in this case and were consistent with diagnostic
2. Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto
criteria of sCJD. Although a subacute onset is typi- A, Heinemann U, Breithaupt M, Varges D, Meissner B,
1,2
cal for sCJD, a stroke-like onset has been recognized Ladogana A, Schuur M, Haik S, Collins SJ, Jansen GH,
as a rare sCJD presentation. 3,4,5 In our case, this severe Stokin GB, Pimentel J, Hewer E, Collie D, Smith P,
neurological picture was entirely driven by a Roberts H, Brandel JP, van Duijn C, Pocchiari M, Begue
C, Cras P, Will RG, Sanchez-Juan P. Updated clinical di-
COVID-19 associated cytokine storm in both the
agnostic criteria for sporadic Creutzfeldt-Jakob disease.
CNS and bloodstream and was fully reversed by im-
Brain 2009; 132 (Pt 10): 2659-2668.
munotherapy, leading to a complete neurological re-
3. Mead S, Rudge P. CJD mimics and chameleons. Pract
covery.
Neurol 2017; 17 (2): 113-121.
The onset of encephalitis symptoms followed an
4. Szabo K, Achtnichts L, Grips E, Binder J, Gerigk L,
asymptomatic COVID-19 pneumonia by at least 10-14
Hennerici M, Gass A. Stroke-like presentation in a case of
days, since positive serum anti-SARS-CoV-2 IgG anti- Creutzfeldt-Jakob disease. Cerebrovasc Dis 2004; 18 (3):
bodies were found in the first week after admission. 251-253.
Overall, our case provides further evidence for an im-
5. Hohler AD, Flynn FG. Onset of Creutzfeldt-Jakob disease
mune-mediated, but likely not antibody-mediated, mimicking an acute cerebrovascular event. Neurology
mechanism of a subtype of COVID-19 associated en- 2006; 67 (3): 538-539.

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