Alzheimers Dementia:合并病理的原发性进行性失语症,神经功能退化更明显

2021-06-03 Freeman MedSci原创

合并病理的原发性进行性失语症,神经功能退化更明显

原发性进行性失语症(PPA)是一种临床痴呆综合征,其特点是最初在理解或表达语言方面存在孤立的缺陷,并将随着时间的推移而下降。它最常与额颞叶变性(FTLD;60%)或阿尔茨海默病(AD;40%)的神经病理学有关。语言症状有已知的变异性,并随着时间的推移出现额外的认知和功能衰退,这一点是目前仍不清楚,而且很难在个人层面上预测。这种模糊性使得人们很难对疾病进展的速度做出现实的预期,从而影响了家庭制定适当的护理计划以应对这些挑战的能力。


初步证据表明,非语义性PPA与FTLD神经病理学相比,其萎缩更广泛,临床和解剖学进展更快,这表明神经病理学的类型可能为理解衰退的速度和预后提供了新视角。这些发现表明,PPA中AD的存在可能与功能能力的不相称的下降有关,但这一点在疑似神经病理学的背景下还没有被探讨过。相反,PPA中不同的功能衰退的存在是通过表型亚型的角度来描述的,并且在某一类型的衰退是否比另一类型更快方面,尚无定论。

University Feinberg School of Medicine的 Stacey Moeller等人的研究,填补这一空白,他们采用前瞻性纵向设计,在24个月的过程中,以临床相关的6个月为间隔,对有和没有AD生物标志物(PPAAβ+和PPAAβ-)的非语义性PPA的功能衰退进行描述和评估。

并使用日常生活活动问卷(ADLQ)测量功能下降,该问卷旨在评估痴呆症患者的日常功能。功能活动的下降是通过总的综合得分和构成综合得分的日常生活活动的六个方面来考察的:自我照顾、家庭护理、就业和娱乐、购物和金钱、旅行和交流。

鉴于PPA中报告的疑似AD病理的萎缩模式更为广泛,因此有利于预测:

有AD生物标志物(PPAAβ+)的参与者在基线和随着时间的推移在日常生活活动方面会比没有AD生物标志物(PPAAβ-)的参与者表现出更大的障碍。

根据以前在没有生物标志物的临床样本中的工作,也可以推测:这两组人在交流之外的功能活动中都会出现下降,特别是随着时间的推移。

他们用线性混合效应模型对17名PPAAβ+和11名PPAAβ-参与者的日常生活活动问卷(ADLQ)评分进行了比较,这些参与者在2年内每隔6个月会有一次疑似潜在的病变。

一个一般线性模型检查了基线时功能下降和皮质厚度之间的关联。

他们发现:各组在人口统计学和失语症严重程度方面没有差异,但在18个月的每个间隔期,PPAAβ+的ADLQ总分和子域分数都比PPAAβ-的明显差(P = 0.015)。

这个研究的重要意义在于:发现了和非AD的神经病理表现相比,非语义性PPA患者(合并AD病理)的功能下降似乎更明显,并破坏了更多的生活活动方面。

原文出处:

Moeller S, Sridhar J, Martersteck A, Coventry C, Kuang A, Zhang H, Weintraub S, Mesulam MM, Rogalski E. Functional decline in the aphasic variant of Alzheimer's disease. Alzheimers Dement. 2021 Apr 8. doi: 10.1002/alz.12331. Epub ahead of print. PMID: 33829622.

 
 
 
 

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    2022-03-10 anminleiryan
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  5. 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  6. 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  7. 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  8. 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objectType=article, channel=null, level=null, likeNumber=32, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=94392, encryptionId=acd4943925f, topicName=进行性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=869698, createdName=ligang4439, createdTime=Fri Jan 07 11:19:36 CST 2022, time=2022-01-07, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1942948, encodeId=8fa41942948fd, content=<a href='/topic/show?id=3e40443166e' target=_blank style='color:#2F92EE;'>#失语症#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=32, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=44316, encryptionId=3e40443166e, topicName=失语症)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=5fc9246, createdName=zhangfenasdf, createdTime=Fri Apr 22 16:19:36 CST 2022, time=2022-04-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1338505, encodeId=8798133850527, content=<a href='/topic/show?id=767711566a9' target=_blank style='color:#2F92EE;'>#MERS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=11566, encryptionId=767711566a9, topicName=MERS)], attachment=null, authenticateStatus=null, createdAvatar=https://thirdwx.qlogo.cn/mmopen/vi_32/71tKRib0PibveVjnEa7tPLMaCzoKicFlKSEOzRTGf4wNvDV54kJyNx1ott1D5Xp5gRu7IwUia77oN0YFdffvQMu6Ug/132, createdBy=9e302500146, createdName=ms49895894946433513521, createdTime=Thu Jun 03 19:19:36 CST 2021, time=2021-06-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1422095, encodeId=302c1422095bc, content=<a href='/topic/show?id=e5f0e42224f' target=_blank style='color:#2F92EE;'>#神经功能#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=34, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=74222, encryptionId=e5f0e42224f, topicName=神经功能)], 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  9. [GetPortalCommentsPageByObjectIdResponse(id=1878376, encodeId=c29818e83760a, content=<a href='/topic/show?id=c157696175' target=_blank style='color:#2F92EE;'>#ERS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6961, encryptionId=c157696175, topicName=ERS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e93e105, createdName=anminleiryan, createdTime=Thu Mar 10 02:19:36 CST 2022, time=2022-03-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1689745, encodeId=004a1689e4564, content=<a href='/topic/show?id=a7c54431571' target=_blank style='color:#2F92EE;'>#失语#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=41, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=44315, encryptionId=a7c54431571, topicName=失语)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=adcc28855380, 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objectType=article, channel=null, level=null, likeNumber=32, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=94392, encryptionId=acd4943925f, topicName=进行性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=869698, createdName=ligang4439, createdTime=Fri Jan 07 11:19:36 CST 2022, time=2022-01-07, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1942948, encodeId=8fa41942948fd, content=<a href='/topic/show?id=3e40443166e' target=_blank style='color:#2F92EE;'>#失语症#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=32, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=44316, encryptionId=3e40443166e, topicName=失语症)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=5fc9246, createdName=zhangfenasdf, createdTime=Fri Apr 22 16:19:36 CST 2022, time=2022-04-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1338505, encodeId=8798133850527, content=<a href='/topic/show?id=767711566a9' target=_blank style='color:#2F92EE;'>#MERS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=11566, encryptionId=767711566a9, topicName=MERS)], attachment=null, authenticateStatus=null, createdAvatar=https://thirdwx.qlogo.cn/mmopen/vi_32/71tKRib0PibveVjnEa7tPLMaCzoKicFlKSEOzRTGf4wNvDV54kJyNx1ott1D5Xp5gRu7IwUia77oN0YFdffvQMu6Ug/132, createdBy=9e302500146, createdName=ms49895894946433513521, createdTime=Thu Jun 03 19:19:36 CST 2021, time=2021-06-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1422095, encodeId=302c1422095bc, content=<a href='/topic/show?id=e5f0e42224f' target=_blank style='color:#2F92EE;'>#神经功能#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=34, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=74222, encryptionId=e5f0e42224f, topicName=神经功能)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=85503680375, createdName=lhlxtx, createdTime=Thu Jun 03 19:19:36 CST 2021, time=2021-06-03, status=1, ipAttribution=)]

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