Objectives As of today, clinical diagnosis of sporadic Creutzfeldt–Jakob disease (sCJD) remains a major challenge and needs to be corroborated by precision biomarkers. Despite moderate sensitivity, periodic electroencephalogram (EEG) abnormalities are of interest due to high specificity. However, their clinical yield could be much improved by a better understanding of their pathophysiological significance, their evolution in time and their overlap with epileptic discharge. Here we illustrate a typical EEG-evolution in sCJD, complicated in the end by prolonged electro-clinical seizures, and discuss the question of blurred boundaries between encephalopathic and epileptic patterns in sCJD. Methods Electro-clinical analysis of five successive video-EEG recordings in a patient with an initially dubious clinical presentation (ophthalmoplegia, preserved cognition, MRI and cerebrospinal fluid inconclusive). Within three months, she developed the full electro-clinical picture of sCJD. The findings are confronted with a review of the literature. Results Early recordings showed a regional encephalopathic pattern with focal slow waves attenuated by stimulation and sleep, localized in close vicinity to asymmetrical enhancement of diffusion signal on MRI. Concomitant to increasing cortical ribboning, EEG abnormalities became periodic, bilateral and finally evolved to prolonged individual electro-clinical seizures. EEG thus had a dual diagnostic role in this patient: early abnormalities helped to orient toward sCJD, late patterns revealed the presence of clinically ambiguous focal seizures. Conclusion Typical EEG changes in sCJD concern 60% of patients and seem to emanate from diverse pathophysiological mechanisms. Abnormalities evolve from early, rather non-specific encephalopathic patterns to unequivocal periodic sharp wave complexes and in 15–21% of cases, to seizures late in the disease. This evolution corresponds to an increasing degree of cortical hyperexcitability, with blurred transitions to non-convulsive status epilepticus. We propose that EEG patterns in sCJD represent diverse pathophysiological mechanisms with fluid boundaries and ultimately express an increasing burden of cortical hyperexcitability.
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