朊蛋白病（creutzfeld jakob disease,CJD）是一类慢性进行性致死性神经系统变性疾病,是继癌症、艾滋病后又一严重威胁人类和动物健康的疾病.虽然该类疾病临床罕见,但应该引起足够的认识,一旦漏诊和误诊容易给患者家庭及社会造成一定的...朊蛋白病（creutzfeld jakob disease,CJD）是一类慢性进行性致死性神经系统变性疾病,是继癌症、艾滋病后又一严重威胁人类和动物健康的疾病.虽然该类疾病临床罕见,但应该引起足够的认识,一旦漏诊和误诊容易给患者家庭及社会造成一定的危害.本科2009年10月至2010年2月共收治2例CJD患者,现报道如下.展开更多
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 w...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.展开更多
文摘朊蛋白病（creutzfeld jakob disease,CJD）是一类慢性进行性致死性神经系统变性疾病,是继癌症、艾滋病后又一严重威胁人类和动物健康的疾病.虽然该类疾病临床罕见,但应该引起足够的认识,一旦漏诊和误诊容易给患者家庭及社会造成一定的危害.本科2009年10月至2010年2月共收治2例CJD患者,现报道如下.
文摘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.
