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Abstract

Creutzfeldt-Jakob disease is a rare neurodegenerative disorder characterized by rapidly progressive dementia and neurological symptoms. While the classic presentation of CJD includes dementia, myoclonus, and specific EEG findings, atypical manifestations can complicate the diagnostic process. This article explores the unique presentation of CJD as nonconvulsive status epilepticus (NCSE), a form of status epilepticus without overt convulsive activity. The atypical nature of this presentation poses significant diagnostic challenges, as it can mimic other conditions. Early recognition of CJD-related NCSE is crucial for appropriate management and counseling. This article highlights the importance of increased awareness and understanding of this unusual clinical phenotype.

Keywords: Creutzfeldt-Jakob disease; Nonconvulsive status epilepticus; Atypical presentation; Diagnostic challenges; Neurodegenerative disorder