De Wever, Centre for Elderly Care, Tilburg, The Netherlands.
Department of Primary and Community Care, Centre for Family Medicine, Geriatric Care and Public Health, Radboud University Medical Centre, Nijmegen, The Netherlands.
J Alzheimers Dis. 2018;63(1):343-351. doi: 10.3233/JAD-170859.
The progression of dementia in people with young-onset dementia (YOD) is relatively unknown.
To investigate the progression of dementia and cognitive decline in the three most common subtypes in YOD and to explore which factors are associated with this course.
The course of dementia was examined in 198 people with YOD. The primary outcomes were cognitive function, as assessed by the Mini-Mental State Examination (MMSE) and dementia severity, as assessed by the Global Deterioration Scale (GDS). Mixed-model analyses were used to explore factors associated with the course of dementia of the diagnostic sub-types.
The mean overall two-year progression of dementia severity was 0.9 GDS points, this was a statistically significant change (p = 0.012) and was not significant different for the three dementia subtypes. The mean overall two-year decline in cognitive function was 1.6 points on the MMSE. The differences in cognitive decline were statistically significant (p = 0.046) among the three diagnosis groups, AD participants showed the greatest decline, of 2.3 points. In addition to lower education (p = 0.010), higher scores on the Neuropsychiatric Inventory (NPI) sub-syndromes psychosis (p < 0.001) and hyperactivity (p = 0.002) were associated with higher rates of cognitive decline. In contrast, higher scores on the NPI affect cluster were associated with lower levels of cognitive decline (p < 0.001).
Different YOD subtypes show different rates of decline in cognitive functioning, and this decline seems less progressive compared to those observed in studies in late-onset AD. Further research is needed to evaluate whether managing neuropsychiatric symptoms can positively influence the decline of cognitive function.
早发性痴呆（YOD）患者的痴呆进展相对未知。
研究 YOD 中最常见的三种亚型的痴呆进展和认知能力下降，并探讨哪些因素与此过程相关。
对 198 名 YOD 患者的痴呆病程进行了检查。主要结局指标是认知功能，采用简易精神状态检查量表（MMSE）评估；痴呆严重程度，采用总体衰退量表（GDS）评估。采用混合模型分析探讨诊断亚型痴呆病程相关的因素。
痴呆严重程度的总体两年进展平均值为 0.9 GDS 点，这是统计学上显著的变化（p=0.012），且在三种痴呆亚型之间无显著差异。MMSE 认知功能的总体两年下降平均值为 1.6 分。三组诊断组之间的认知下降差异具有统计学意义（p=0.046），AD 患者下降幅度最大，为 2.3 分。除了受教育程度较低（p=0.010）外，神经精神量表（NPI）亚综合征精神病（p<0.001）和多动（p=0.002）评分较高与认知下降率较高相关。相反，NPI 情感障碍评分较高与认知下降水平较低相关（p<0.001）。
不同的 YOD 亚型表现出不同的认知功能下降率，与在迟发性 AD 研究中观察到的相比，这种下降似乎不那么进行性。需要进一步研究来评估是否管理神经精神症状可以对认知功能的下降产生积极影响。
