Objective: To assess cognitive function in variant Creutzfe- ldt-Jakob disease (vCJD). We describe the neuropsychological profiles of 10 c ases and compare these data with cross sectional data obtained from patients wit h histologically confirmed sporadic CJD and cases with inherited prion disease w ith confirmed mutations in the prion protein gene. Methods: Patients referred to the Specialist Cognitive Dis orders Clinic at the National Hospital for Neurology and Neurosurgery and the National Prion Clinic at St Mary’s Hospital, London for further investigation o f suspected CJD were recruited into the study. The neuropsychological test batte ry evaluated general intelligence, visual and verbal memory, nominal skills, lit eracy skills, visual perception and visuospatial functions, and visuospatial and executive function. Results: The results indicate that moderate to severe cogni tive decline is a characteristic feature of vCJD. Specifically,verbal and visual memory impairments and executive dysfunction were pervasive in all disease grou ps. Nominal skills were impaired in variant and sporadic CJD, significantly so w hen compared with the inherited prion disease group. Perceptual impairment was l ess frequent in the vCJD group than in the sporadic and inherited groups. Conclu sion: This study confirms the occurrence of generalised cognitive decline in pat ients with vCJD. Although decline in cognitive function ultimately affects all d omains, there is a suggestion that some components of visual perception may be s pared in vCJD. The results also suggest that nominal function may be preserved i n some cases with inherited prion disease. Objective: To assess cognitive function in variant Creutzfe-ldt-Jakob disease (vCJD). We describe the neuropsychological profiles of 10 c ases and compare these data with cross sectional data obtained from patients wit h histologically confirmed sporadic CJD and cases with inherited prion disease Methods: Patients referred to the Specialist Cognitive Dis orders Clinic at the National Hospital for Neurology and Neurosurgery and the National Prion Clinic at St Mary’s Hospital, London for further investigation of suspected CJD were recruited into the study. The neuropsychological test batte ry evaluated general intelligence, visual and verbal memory, nominal skills, lit eracy skills, visual perception and visuospatial functions, and visuospatial and executive function. Results: The results indicating that moderate to severe cognizance decline is a characteristic feature of vCJD. Specifically, verbal and visual memory impairments and exec utive dysfunction were pervasive in all disease grou ps. Nominal skills were impaired in variant and sporadic CJD, significantly so w hen compared with the inherited prion disease group. Perceptual impairment was l ess frequent in the vCJD group than in the sporadic and inherited groups. Conclu sion: This study confirms the occurrence of generalized cognitive decline in pat ients with vCJD. The results in generalized cognitive decline in pat ients with vCJD. The results also suggest that some components of visual perception may be s pared in vCJD. The results also suggest that nominal function may be preserved in some cases with inherited prion disease.