Abstract

Sporadic Creutzfeldt–Jakob Disease (sCJD) is a rapidly progressive neurodegenerative disorder usually affecting people between 60 and 70 years old, with only anecdotal cases presenting at 90 years or older. The clinical phenotype of sCJD is highly variable. Diagnosis of sCJD should be considered in the differential diagnosis of rapidly evolving ataxic or dementing syndromes with or without epileptic seizures, regardless of the patient age. While the recognition of atypical phenotypes in subject 90 years or older can provide additional diagnostic challenge, it must be underlined that neuropathology is still the “gold standard” for sCJD diagnosis.