A prospective 14-year longitudinal follow-up of dementia in persons with Down syndrome
A prospective 14-year longitudinal follow-up of dementia in persons with Down syndrome
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DOI：
10.1111/jir.12074
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发表时间：
2014-01-01
影响因子：
3.6
通讯作者：
Mulryan, N.
中科院分区：
文献类型：
--
作者：
McCarron, M.;McCallion, P.;Mulryan, N.
背景旨在检查唐氏综合症患者的痴呆特征、发病年龄和相关合并症。方法对 77 名 35 岁及以上的唐氏综合症患者进行纵向随访。痴呆症的诊断是使用修订后的国际疾病分类第十次修订版 (ICD-10) 标准以及客观和基于信息的测试相结合来确定的。认知测试包括严重障碍测试和唐氏综合症精神状态检查；使用日常生活技能问卷来测量适应性行为。智障人士痴呆问卷 (DMR) 于 2005 年添加到测试组中，本研究包括该仪器的后续数据。结果在 14 年期间，平均诊断年龄为 55.41 岁 (SD=7.14)，处于先前报告的估计的较高范围内 (51-56 岁)，诊断后中位生存期为 7 年。样本中患有痴呆症的人明显比没有痴呆症的人年龄大。痴呆症的存在也与癫痫和感觉障碍有关。在诊断前，DMR 似乎对跟踪症状随时间的变化最为敏感。 结论 这些数据证实了先前报告的唐氏综合症患者患痴呆症的高风险水平，以及用于跟踪衰退和帮助确认诊断的工具的价值，即使是对于患有严重智力障碍的人。
BackgroundTo examine dementia characteristics, age at onset and associated comorbidities in persons with Down syndrome.MethodSeventy-seven people with Down syndrome aged 35 years and older were followed longitudinally. The diagnosis of dementia was established using the modified International Classification of Diseases, Tenth Revision (ICD-10) criteria and a combination of objective and informant-based tests. Cognitive tests included the Test for Severe Impairment and the Down Syndrome Mental Status Examination; adaptive behaviour was measured using the Daily Living Skills Questionnaire. The Dementia Questionnaire for Mental Retarded Persons (DMR) was added to the test battery in 2005 and this study includes follow-up data for this instrument.ResultsOver the 14-year period the average age of diagnosis at 55.41 years (SD=7.14) was in the higher range of previously reported estimates (51-56 years) and a median survival of 7 years after diagnosis. Persons with dementia in the sample were significantly older than persons without dementia. The presence of dementia was also associated with epilepsy and sensory impairments. Among instruments the DMR appeared most sensitive to tracking change in symptoms over time before diagnosis.ConclusionThe previously reported high risk levels for dementia among people with Down syndrome was confirmed in these data as was the value of the instruments utilised in tracking decline and helping to confirm diagnosis even in persons with severe intellectual disability.