Introduction
Voltage-gated potassium channel-complex antibodies (VGKC-cAbs) encephalitis, a treatable autoantibody encephalopathy, has been previously reported to clinically mimic sporadic Creutzfeldt-Jacob disease (sCJD). Among available clinical clues to distinguish them, periodic sharp wave complexes (PSWC), a typical finding in sCJD, have never been reported in association with VGKC-cAbs encephalitis.
Case presentation
A 76 years old man was transferred to a tertiary neurology center with a clinical history of six-month weight loss, cognitive disturbance and non specific generalized weakness. He had two seizures the month before transfer and then evolved to severe encephalopathy, requiring mechanical ventilation. PSWC every 1–2 seconds over slowed background were found on EEG, and MRI showed cerebellar and bifrontal cortical T2/FLAIR/DWI hypersignal without restricted diffusion on ADC mapping. Pancorporal PET-scan was negative. An immunotherapy trial did not improve the patient condition. Therefore, he died after life support withdrawal. Brain autopsy revealed mononuclear neocortex infiltrate without significant spongiosis, and the anti-VGKC test showed a seropositivity of 336 pmol/L (normal: 0–31), three month after the patient deceased.
Conclusion
This is the first reported case of VGKC-cAbs encephalitis associated with PSWC on EEG, which further confuse the differential diagnosis with sCJD. However, the cortical DWI hypersignal without restriction seem to remain a way to discriminate these two entities appropriately, when present. These clues are of paramount importance since VGKC-cAbs encephalitis is a treatable disease.