ABSTRACT

Introduction

Having a diagnosis of Down syndrome (DS) is associated with intellectual disability (ID), pervasive developmental disorders and Alzheimer’s dementia (AD). The association between these conditions has not been well evaluated. This paper looks to examine the current evidence pertaining to the relationship between dementia in people with DS and severity of ID and the presence of pervasive developmental disorders.

Methods

A scoping review using PRISMA guidance was undertaken. Medline, Cochrane database, NHS evidence, Trial registers and Open Grey were searched in December 2018 and an updated search was completed in July 2020. Three search strategies were used to retrieve articles relating to DS, dementia, pervasive developmental disorders (including autism, autism spectrum disorder (ASD) and attention-deficit hyperactivity disorder (ADHD)) and severity of ID. Studies were included if they met the pre-defined inclusion criteria of investigating an association between autism/ASD, ADHD, or severity of ID and the development of dementia in people with DS. Studies were excluded if they did not include primary data, if the population included non-Down causes of ID, or if no specific outcome measure related to comorbid autism/ASD, ADHD, or severity of ID and dementia in people with a diagnosis of DS were reported. There were no exclusions related to study design. Papers were assessed for quality using the Mixed Methods Appraisal Tool (MMAT).

Results:

The search identified 15 papers, publishing results from 12 studies, relating to severity of ID, DS and dementia. No papers were identified relating to pervasive developmental disorders, DS and dementia. There is limited evidence on how severity of ID impacts on the presentation, diagnosis, management or prognosis of dementia in people with DS. However, no evidence was found on comorbid pervasive developmental disorders, DS and dementia.

Conclusion:

This paper has identified multiple areas for future research. There is an urgent need for longitudinal studies into the presentation, development and progression of dementia in people with DS ensuring the severity of ID and comorbid pervasive developmental conditions are captured regularly to understand their influence on the dementia etiology and outcome.

中文翻译：

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智障和/或神经发育障碍的严重程度是否会影响唐氏综合症患者痴呆症的发作？

摘要

介绍

诊断为唐氏综合症（DS）与智力残疾（ID），普遍的发育障碍和阿尔茨海默氏痴呆症（AD）有关。这些条件之间的关联尚未得到很好的评估。本文旨在研究与DS痴呆症和ID严重程度以及普遍存在的发育障碍之间的关系有关的最新证据。

方法

使用PRISMA指南进行了范围界定审查。在2018年12月对Medline，Cochrane数据库，NHS证据，试验注册和开放灰色进行了搜索，并于2020年7月完成了更新搜索。使用了三种搜索策略来检索与DS，痴呆，普遍性发育障碍（包括自闭症，自闭症）相关的文章频谱障碍（ASD）和注意力缺陷多动障碍（ADHD））和ID的严重程度。如果符合预定的纳入标准，即研究自闭症/ ASD，ADHD或ID的严重程度与DS患痴呆症之间的关系，则纳入研究。如果研究不包括主要数据，人群中包括非唐氏病的ID病因，或者没有与自闭症/ ASD，ADHD，报告了诊断为DS的人的ID或痴呆严重程度。没有与研究设计相关的排除。使用混合方法评估工具（MMAT）对论文进行质量评估。

结果：

搜索确定了15篇论文，发表了12项研究的结果，这些研究涉及ID，DS和痴呆的严重程度。没有发现与广泛性发育障碍，DS和痴呆有关的论文。关于ID严重程度如何影响DS患者痴呆症的表现，诊断，管理或预后的证据有限。但是，没有发现关于合并症的普遍性发育障碍，DS和痴呆症的证据。

结论：

本文确定了未来研究的多个领域。迫切需要对DS患者痴呆症的表现，发展和进展进行纵向研究，以确保定期捕获ID的严重程度和合并的普遍性发展状况，以了解其对痴呆病因和结局的影响。