Sporadic Creutzfeldt-Jakob disease mimicking refractory non-convulsive status epilepticus: Diagnostic pitfalls of EEG interpretation

Background Sporadic Creutzfeldt–Jakob disease (sCJD) is a rare and uniformly fatal prion disease that may mimic non-convulsive status epilepticus (NCSE) in its early stages because of overlapping clinical and electroencephalographic features. Distinguishing these conditions is essential to avoid diagnostic delay and inappropriate treatment. Case Presentation We report the case of a 60-year-old woman admitted with impaired consciousness who was initially diagnosed with refractory NCSE. Despite treatment with benzodiazepines and multiple antiseizure medications, no clinical improvement was observed. Electroencephalography demonstrated periodic sharp wave complexes (PSWCs), which transiently disappeared following midazolam administration without recovery of consciousness. Brain magnetic resonance imaging revealed bilateral cortical and basal ganglia diffusion hyperintensities, while cerebrospinal fluid analysis was positive for the 14-3-3 protein. Together, these findings supported the diagnosis of probable sporadic Creutzfeldt–Jakob disease. Conclusion This case illustrates the diagnostic challenge of differentiating sCJD from NCSE. Transient suppression of PSWCs after benzodiazepine administration in the absence of clinical improvement should prompt consideration of prion disease, although this finding is not specific and should be interpreted within the overall clinical context. Careful integration of clinical presentation, electroencephalographic findings, neuroimaging, and cerebrospinal fluid biomarkers is essential for timely diagnosis and to avoid unnecessary escalation of antiseizure therapy.