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Proceedings SNO “Percorsi clinici in Neuroscienze”

A B

Figure 1. Representative EEG epochs showing left-sided lateralized periodic discharges with associated myoclonus on day 1 (A)
and generalized periodic discharges on day 7 (B). EMG with right flexor carpi surface electromyography electrode.

bands were present in both CSF and serum (pattern ment, a differential diagnosis between acute-onset
type 4). Onconeural antibodies (GAD-65, Zic4, Tr, sCJD and autoimmune encephalitis associated with
SOX1, Ma2, Ma1, amphiphysin, CRMP5, Hu, Yo, COVID-19 was hypothesized.
Ri), GAD-65 and neural surface antigens antibodies Further diagnostic tests were performed on the CSF
(VGKC, LGI1, CASPR2, DPPX, NMDAr, AMPA1- and serum samples: 14.3.3 protein was positive on a
2, mGluR3, GABAb1, VGCC) were absent in serum CSF sample from day 10; nonetheless, RT-QuIC
and CSF. He was initially treated with intravenous di- analysis did not show any positive seeding activity due
azepam followed by intravenous antiepileptic drugs the presence of prion; re-assessment of the CSF and
(valproate, levetiracetam, lacosamide), without clini- serum samples from day 1 showed very high levels of
cal benefit. The day after admission, the level of con- IL-6 in the CSF (299 pg/mL) compared to serum (20
sciousness decreased to GCS 7 (no eyes opening, no
pg/mL), elevated levels of IL-23 in both CSF (81
verbal response, localizing pain on the left, no motor pg/mL) and serum (333 pg/mL) and elevated levels of
response on the right) and acute respiratory failure IL-31 in both CSF (20 pg/mL) and serum (424
developed, requiring intubation and transfer to the
pg/mL).
Intensive Care Unit. The patient was treated with high-dose intravenous
Continuous EEG monitoring showed evolution of the methylprednisolone (1000 mg/day for 5 days), imme-
EEG pattern to generalized periodic epileptiform dis-
diately followed by intravenous immunoglobulins (0.4
charges at 1 Hz (Figure 1B), which were transiently
g/kg/day for 5 days). A clear EEG improvement was
abolished during two cycles of anesthetics (propofol-
observed during the last day of immunoglobulin infu-
midazolam for 24 hours and ketamine-midazolam for
sion, with disappearance of generalized periodic dis-
48 hours), but relapsed after withdrawal of anesthet-
charges. Anesthetics were withdrawn and antiepileptic
ics. Add-on perampanel had no effect on either EEG
drugs were reduced, followed by gradual improvement
or clinical picture. On day 3, a first brain MRI was
normal. Seven days later (on day 10) a second brain of consciousness with no relapse of seizures or my-
MRI showed signal hyperintensity of the cortical rib- oclonus. A third brain MRI, performed 7 weeks after
bon of the left perisylvian regions (insula, middle hospital admission, showed disappearance of the pre-
frontal gyrus, inferior parietal lobule, and superior viously detected cortical abnormalities (Figure 2B).
temporal gyrus) and bilateral cingulate gyrus on DWI In the following weeks, the patient regained a full
sequences, without concomitant reduction on the functional status, including cognitive abilities, and
ADC map and with subtle hyperintensities on FLAIR was discharged home. At the follow-up visit 6 months
sequences (Figure 2A). later, his neurological examination was unremarkable
Considering MRI evolution, EEG showing periodic and no further seizure occurred.
sharp wave complexes and refractoriness to treat-

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