Loi Samantha M, Pijnenburg Yolande, Velakoulis Dennis
Department of Neuropsychiatry, Royal Melbourne Hospital.
Department of Psychiatry, University of Melbourne, Parkville, VIC, Australia.
Curr Opin Psychiatry. 2023 Mar 1;36(2):126-133. doi: 10.1097/YCO.0000000000000843. Epub 2022 Dec 28.
Young-onset dementia (YOD) refers to a dementia for which symptom onset occurs below the age of 65. This review summarizes the recent literature in this area, focusing on updates in epidemiology, diagnosis and service provision.
In the last year, internationally, the prevalence of YOD was reported as 119 per 100 000, but this may vary according to population types. Although the commonest causes of YOD are Alzheimer's disease (AD) and frontotemporal dementia (FTD), there is increasing recognition that YOD is diagnostically and phenotypically broader than AD and FTD. YOD may be due to many other diseases (e.g. Huntington's disease, vascular dementia) whereas accumulation of the same protein (e.g. amyloid protein) may lead to different phenotypes of Alzheimer's disease (such as posterior cortical atrophy and behavioural-variant/frontal-variant AD). This heterogeneity of phenotypic presentation is also seen in YOD due to known genetic mutations. Biomarkers such as plasma and cerebrospinal fluid proteins, neuroimaging and genetics have shown promise in the early identification of YOD as well as providing further understanding behind the overlap between psychiatric and neurodegenerative conditions occurring in younger people. The management of YOD needs to consider age-specific issues for younger people with dementia and their family networks together with better integration with other health services such as aged, disability and improved access to services and financial assistance.
These findings emphasize the need for early identification and appropriate age-specific and person-centred management for people with young-onset dementia.
早发性痴呆(YOD)是指症状出现年龄低于65岁的痴呆。本综述总结了该领域的最新文献,重点关注流行病学、诊断和服务提供方面的更新。
去年,国际上报道的YOD患病率为每10万人中有119例,但这可能因人群类型而异。虽然YOD最常见的病因是阿尔茨海默病(AD)和额颞叶痴呆(FTD),但人们越来越认识到YOD在诊断和表型上比AD和FTD更广泛。YOD可能由许多其他疾病引起(如亨廷顿舞蹈病、血管性痴呆),而相同蛋白质(如淀粉样蛋白)的积累可能导致阿尔茨海默病的不同表型(如后部皮质萎缩和行为变异型/额叶变异型AD)。由于已知的基因突变,这种表型表现的异质性在YOD中也可见。血浆和脑脊液蛋白、神经影像学和遗传学等生物标志物在YOD的早期识别方面显示出前景,同时也有助于进一步理解年轻人中发生的精神疾病和神经退行性疾病之间的重叠。YOD的管理需要考虑年轻痴呆患者及其家庭网络的特定年龄问题,以及与老年、残疾等其他卫生服务更好地整合,并改善服务和经济援助的获取。
这些发现强调了对早发性痴呆患者进行早期识别以及采取适当的特定年龄和以患者为中心的管理的必要性。