唐氏综合征所致痴呆

定义

唐氏综合征所致痴呆症是一种神经退行性疾病，与淀粉样前体蛋白（APP）的异常增生和积累导致β-淀粉状蛋白块和tau缠结形成有关。APP 基因表达增加的原因是其位于 21 号染色体上，而唐氏综合症患者的 21 号染色体是异常的三倍体。认知缺陷和神经病理学特征与阿尔茨海默病相似。该病通常在患者四十岁后发病，功能逐渐减退，50% 或更多的唐氏综合征患者受到影响。

Dementia due to Down syndrome is a neurodegenerative disorder related to the impact of abnormal increased production and accumulation of amyloid precursor protein (APP) leading to formation of beta-amyloid plaques and tau tangles. APP gene expression is increased due to its location on chromosome 21, which is abnormally triplicated in Down syndrome. Cognitive deficits and neuropathological features are similar to those observed in Alzheimer disease. Onset is typically after the fourth decade of life with a gradual decline in functioning, and may impact 50% or more of individuals with Down syndrome.

索引词 可用于检索该条目的同义词与别名

• 唐氏综合征所致痴呆

编码备注 Coding note

这些分类不可用作主要编码。当需要标识分类于他处的疾病引起的痴呆时，可将这些编码作为补充或附加编码。 对于多种病因引起的痴呆，对所有适用的病因进行编码。

This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere. When dementia is due to multiple aetiologies, code all that apply.