School of Nursing & Midwifery, Trinity College Dublin, Dublin, Ireland.
J Intellect Disabil Res. 2014 Jan;58(1):61-70. doi: 10.1111/jir.12074. Epub 2013 Jul 31.
To examine dementia characteristics, age at onset and associated comorbidities in persons with Down syndrome.
Seventy-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.
Over 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.
The 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.
研究唐氏综合征患者痴呆的特征、发病年龄和相关合并症。
对 77 名年龄在 35 岁及以上的唐氏综合征患者进行纵向随访。采用改良后的国际疾病分类第十版（ICD-10）标准和客观与信息源测试相结合的方法诊断痴呆。认知测试包括严重损害测试和唐氏综合征精神状态检查；采用日常生活技能问卷测量适应行为。2005 年，在测试组合中加入了智障人士痴呆问卷（DMR），本研究包括该测试工具的随访数据。
在 14 年的时间里，平均诊断年龄为 55.41 岁（SD=7.14），处于先前报告的估计值（51-56 岁）的较高范围，且诊断后中位生存时间为 7 年。样本中痴呆患者的年龄明显大于非痴呆患者。痴呆的存在也与癫痫和感觉障碍有关。在这些工具中，DMR 似乎在诊断前跟踪症状变化方面最敏感。
这些数据证实了唐氏综合征患者痴呆风险高的先前报告，也证实了在跟踪衰退和帮助确诊，甚至在智力严重残疾患者中，使用这些工具的价值。
