JNNP:血浆胶质纤维酸性蛋白和神经丝轻链是衡量原发性进行性失语症患者病情严重程度的指标

2020-11-28 MedSci原创 MedSci原创

语义变异性原发性进行性失语症(svPPA)是一种神经退行性疾病,其特征在于概念知识的丧失,通常表现为单词查找困难,单个单词理解能力受损和颞叶局灶性萎缩。它是额颞叶痴呆(FTD)的一种类型通常与TDP-

从伦敦大学学院FTD队列研究的64位连续入选的受试者中收集血浆样品:28位符合svPPA1诊断标准的受试者和36位健康对照。两组之间的年龄或性别均无统计学差异(t检验,p = 0.934,Fisher精确检验,p = 1.000)。参加者接受了标准化的临床和认知评估(表1),其中包括语义知识的两种测量方法,即韦氏智力量表(WASI)词汇量表子测验和英国图片词汇量表。通过Siemens Trio或Prisma 3T扫描仪对大脑进行了3D T1加权MRI,进行了55次扫描,计算颞叶灰质体积(使用先前描述的自动分割技术计算左半球和右半球的总和。 使用Neurology 4-Plex A试剂盒对GFAP和NfL进行了测定。
 

与临床诊断相一致,svPPA组在两项语义知识测试(WASI词汇表,z=6.20,p<0.001;BPVS,z=6.35,p<0.001)上均明显受损,且颞叶体积较低(t=12.79,p<0.001)。在svPPA组(rho=0.53,p=0.004)和对照组(rho=0.51,p=0.002)中,GFAP和NfL水平均显著相关。在svPPA组,NfL增加与语义损害程度无关(NfL:WASI词汇表,rho=−0.55,p=0.004;BPVS,rho=−0.56,p=0.003;GFAP:WASI词汇表,rho=−0.22,p=0.291;BPVS,rho=−0.26,p=0.204)。NfL和GFAP浓度均与颞叶容积呈负相关(NfL,rho=−0.47,p=0.033;GFAP,rho=−0.58,p=0.006)。

与GFAP相比,NfL的作用范围更大,但GFAP和NfL均可识别svPPA的疾病严重程度。与其他神经退行性疾病相一致,其水平的降低很可能被证明在未来的临床试验中显示出治疗效果。
 
Heller CChan EFoiani MS, et al Plasma glial fibrillary acidic protein and neurofilament light chain are measures of disease severity in semantic variant primary progressive aphasia
 
 
 
 
 
 
 
 
 
 

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  5. 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  6. 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  7. 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GetPortalCommentsPageByObjectIdResponse(id=903168, encodeId=fedc9031688a, content=学习了,涨知识了!, beContent=null, objectType=article, channel=null, level=null, likeNumber=75, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/ajNVdqHZLLDM9swhbgTo7nzqRKzMmzckmEoeTyLQVDCgjqsicjyhxPs0vPoYU6GsSYbCjSkNHicJkz3NL0a0EbUMVdNjkctXI6FocpGKaVr9E/0, createdBy=29392048316, createdName=ms2000001051330459, createdTime=Sat Nov 28 23:15:10 CST 2020, time=2020-11-28, status=1, ipAttribution=)]
  8. 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GetPortalCommentsPageByObjectIdResponse(id=903168, encodeId=fedc9031688a, content=学习了,涨知识了!, beContent=null, objectType=article, channel=null, level=null, likeNumber=75, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/ajNVdqHZLLDM9swhbgTo7nzqRKzMmzckmEoeTyLQVDCgjqsicjyhxPs0vPoYU6GsSYbCjSkNHicJkz3NL0a0EbUMVdNjkctXI6FocpGKaVr9E/0, createdBy=29392048316, createdName=ms2000001051330459, createdTime=Sat Nov 28 23:15:10 CST 2020, time=2020-11-28, status=1, ipAttribution=)]
  9. [GetPortalCommentsPageByObjectIdResponse(id=1976646, encodeId=b09d19e664699, content=<a href='/topic/show?id=acd4943925f' target=_blank style='color:#2F92EE;'>#进行性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=43, 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=Mon Apr 12 17:59:56 CST 2021, time=2021-04-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1942947, encodeId=1d7e194294e27, 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=40, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=44316, encryptionId=3e40443166e, topicName=失语症)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=5fc9246, 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    2020-11-28 12245f72m65暂无昵称

    学习了

    0

  10. 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GetPortalCommentsPageByObjectIdResponse(id=903168, encodeId=fedc9031688a, content=学习了,涨知识了!, beContent=null, objectType=article, channel=null, level=null, likeNumber=75, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/ajNVdqHZLLDM9swhbgTo7nzqRKzMmzckmEoeTyLQVDCgjqsicjyhxPs0vPoYU6GsSYbCjSkNHicJkz3NL0a0EbUMVdNjkctXI6FocpGKaVr9E/0, createdBy=29392048316, createdName=ms2000001051330459, createdTime=Sat Nov 28 23:15:10 CST 2020, time=2020-11-28, status=1, ipAttribution=)]
    2020-11-28 ms2000001051330459

    学习了,涨知识了!

    0

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额颞叶痴呆(FTD)是一种复杂的神经退行性疾病,涵盖一系列症状。行为异常,语言功能障碍,认知缺陷和运动障碍综合形成了FTD的典型症状,但据报道,许多其他症状也经常被忽略,包括疼痛感的改变。一研究的论文

FDA授予AL101快速通道资格,以治疗额颞叶痴呆

临床阶段生物技术公司Alector是免疫神经病学的先驱,今日宣布,美国食品药品监督管理局(FDA)已授予AL101快速通道资格,用于治疗具有致病性前颗粒蛋白基因突变的额颞叶痴呆(FTD-GRN)。

Nat Neurosci:渐冻症和额颞叶痴呆患者中C9ORF72相关的协同致病机制

编码预测鸟嘌呤交换因子C9ORF72的六核苷酸扩增是肌萎缩性脊髓侧索硬化症(ALS)和前颞叶痴呆(FTD)最常见的遗传原因。

Lancet Neurol:影响额颞叶痴呆患者发病及死亡年龄的遗传因素研究

研究表明,遗传额颞叶痴呆患者在发病和死亡时的年龄受遗传群体的影响,特别是MAPT突变,受特定突变和家庭成员身份的影响

JAMA Neurol:成人结节性硬化综合征与额颞叶痴呆相关性研究

成人结节性硬化综合征与额颞叶痴呆临床表现重叠。研究结果支持结节性硬化相关神经精神障碍和额颞叶痴呆之间可能存在的临床连续性,并强调了神经发育和退行性疾病过程之间潜在的病理生理联系

Neurology:遗传性额颞叶痴呆在出现症状前脑室系统就在慢慢的扩大

本研究旨在评级遗传性额颞叶痴呆(FTD)脑室系统扩张的时间进程,识别出现症状前FTD突变携带者脑室扩张的发病时间及发病率。