Characterising the uncommon corticobasal syndrome presentation of sporadic Creutzfeldt-Jakob disease

Abstract

Background: Corticobasal syndrome (CBS), which encompasses cortical sensory loss, alien limb, bradykinesia, rigidity, limb apraxia and dystonia, is the classic presentation of corticobasal degeneration (CBD). It may occur in other neurodegenerative disorders including sporadic Creutzfeldt-Jakob disease (sCJD). Current CBD diagnostic criteria outline features of CBS but fail to distinguish CBD from other causative pathologies. Objectives: To characterise the CBS presentation of sCJD (sCJD-CBS) in the context of existing CBD diagnostic criteria. Method: Data of two new cases of sCJD-CBS and seven patients identified from the Australian National Creutzfeldt-Jakob Disease Registry database was r..