A prospective 20-year longitudinal follow-up of dementia in persons with Down syndrome
A prospective 20-year longitudinal follow-up of dementia in persons with Down syndrome
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DOI：
10.1111/jir.12390
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发表时间：
2017-09-01
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.
目的探讨唐氏综合征患者的痴呆特征、发病年龄和相关合并症。方法对1996 ~ 2015年年龄在35岁及以上的唐氏综合征患者77例进行随访。使用修改后的ICD 10标准以及客观和基于信息的测试相结合来确定痴呆的诊断。认知测试包括严重损伤测试和唐氏综合症精神状态检查；适应性行为是通过日常生活技能问卷来衡量的，自2005年以来，智障人士痴呆症问卷的数据已经可用。结果在20年期间，97.4%（77人中75人）发生痴呆，平均痴呆诊断年龄为55岁（SD = 7.1，中位数= 56岁）。临床痴呆与认知和功能下降以及癫痫发作活动有关。患痴呆症的风险从50岁的23%增加到65岁及以上的80%。不同的ID水平没有差异。结论先前报道的唐氏综合症患者患痴呆的高风险水平在该数据中得到了证实，与晚发性癫痫的关系也是如此。进一步强调了用于跟踪下降和帮助确认诊断的工具的价值。
Goal To examine dementia characteristics, age at onset and associated co-morbidities in persons with Down syndrome.Method A total of 77 people with Down syndrome aged 35 years and older were followed up from 1996 to 2015. The diagnosis of dementia was established using the modified 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, and data from the Dementia Questionnaire for People with Intellectual Disabilities have been available since 2005.Results Over the 20-year period, 97.4% (75 of 77) persons developed dementia with a mean age of dementia diagnosis of 55 years (SD = 7.1, median = 56 years). Clinical dementia was associated with cognitive and function decline and seizure activity. Risk for dementia increased from 23% in those aged 50 years to 80% in those aged 65 years and above. There were no differences by level of ID.Conclusion The previously reported high risk levels for dementia among people with Down syndrome were confirmed in this data as was the relationship with late onset epilepsy. The value of the instruments utilised in tracking decline and helping to confirm diagnosis is further highlighted.