Cortical Ribboning and Periodic EEG Discharges in Probable Sporadic Creutzfeldt-Jakob Disease
Abstract
Raphael Zimmermann, Sheila Zimmermann Kibrit and Allan Zimmermann
Background: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive and fatal prion disease characterized by cognitive decline and variable neurological manifestations.
Case Presentation: We report a 62-year-old woman who developed progressive memory impairment followed by severe neurological deterioration, diffuse myoclonus, and pyramidal and extrapyramidal signs. Brain magnetic resonance imaging (MRI) demonstrated diffuse bilateral cortical signal abnormalities with restricted diffusion and basal ganglia involvement. Electroencephalography (EEG) subsequently revealed generalized periodic sharp-wave complexes. Prion- specific cerebrospinal fluid (CSF) testing was unavailable.
Conclusion: The combination of rapidly progressive cognitive impairment, characteristic neurological manifestations, MRI abnormalities, and typical EEG findings fulfilled diagnostic criteria for probable sCJD. The patient received palliative care and died during the same month. This case highlights the complementary diagnostic value of MRI and EEG in suspected sCJD, particularly when prion-specific biomarkers are unavailable.