Acta Neuropathologica: 食管路易病理学与路易体病进展的相关性:日本尸检病例队列研究

2020-11-29 MedSci原创 MedSci原创

路易体病(Lewy body disease, LBD)是一系列进行性神经退行性疾病,其特征是路易小体和轴突广泛分布于中枢和外周神经系统(CNS、PNS)。

路易体病(Lewy body disease, LBD)是一系列进行性神经退行性疾病,其特征是路易小体和轴突广泛分布于中枢和外周神经系统(CNS、PNS)。临床诊断包括帕金森病(PD)、路易体痴呆或单纯自主神经功能衰竭。所有类型的LBD都伴有非运动症状(NMSs),包括便秘等胃肠功能紊乱。它与肠神经系统(ENS)路易体相关α-突触核细胞病(路易病理学)的关系正引起人们的关注,因为它可以先于运动症状出现。为了阐明ENS-Lewy病理学在疾病进展中的作用,本文利用日本老龄化研究脑库进行了一项临床病理学研究。

方法:518例患者被纳入研究。通过尸检检查CNS和PNS的路易病理学,包括以食管下段为代表的ENS。简单地说,在解剖过程中,首先在矢状面上解剖大脑半球、小脑和脑干,以分离两个半球:一个被冷冻,另一个固定。然后将冰冻侧的大脑切成8毫米的切片:大脑半球在冠状面,小脑在矢状面,脑干在水平面。PNS检查如下。在最大切片上切取心脏和肾上腺水平的6个交感神经节和肾上腺周围脂肪组织。从左心室前壁获得跨壁心肌组织。下段食道和皮肤被解剖成2厘米长,粘膜至外膜或表皮至皮下脂肪组织分为两段。PNS和部分冰冻脑标本,包括额叶和颞极、顶叶(顶叶内沟)、初级视皮层、后海马、杏仁核、小脑(包括齿状核)等在快速冷冻前被解剖成小于2cm×1.5cm的块状物并用4%多聚甲醛(PFA)固定48h。

结果显示,1/3的老年人(178例,34%)表现为路易病理,其中78例(43.8%)表现为食管病变。食管壁中,奥尔巴赫丛(41.6%)最易感病,其次是外膜(33.1%)和迈斯纳丛(14.6%)。食管路易病理与便秘等自主神经功能衰竭显著相关(p<0.0001),在PNS区域中,与LBD进展相关性最大(r=0.95,p<0.05)。

综上所述,本研究为LBD消化功能障碍的发生提供了病理基础,提示食管路易病理是LBD的一个预测因子。

Tanei, Zi., Saito, Y., Ito, S. et al. Lewy pathology of the esophagus correlates with the progression of Lewy body disease: a Japanese cohort study of autopsy cases. Acta Neuropathol (2020). https://doi.org/10.1007/s00401-020-02233-8

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    2020-12-13 longerg
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    2021-09-30 yb6560
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    2021-05-26 windight
  5. 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  6. 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  7. 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status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1437595, encodeId=fed1143e595a7, content=<a href='/topic/show?id=0e311010591a' target=_blank style='color:#2F92EE;'>#食管#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=101059, encryptionId=0e311010591a, topicName=食管)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Tue Dec 01 09:35:30 CST 2020, time=2020-12-01, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1473512, encodeId=04cf14e3512b2, content=<a href='/topic/show?id=4fd1e102257' target=_blank style='color:#2F92EE;'>#病理学#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, 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    2020-12-01 zhouqu_8
  8. 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    2020-12-01 zhouqu_8
  9. 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topicList=[TopicDto(id=71022, encryptionId=4fd1e102257, topicName=病理学)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c8ce7226273, createdName=jktdtl, createdTime=Tue Dec 01 09:35:30 CST 2020, time=2020-12-01, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=903498, encodeId=69379034984f, content=我也是第一次听说这个病。, beContent=null, objectType=article, channel=null, level=null, likeNumber=80, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201120/5008534db22c474a844fbd107ff83af2/d3e20fc133b542208ff15e7f2b575329.jpg, createdBy=c7015311102, createdName=Jessie Zhang, createdTime=Mon Nov 30 10:24:18 CST 2020, time=2020-11-30, status=1, ipAttribution=)]
    2020-12-01 jktdtl
  10. 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status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1437595, encodeId=fed1143e595a7, content=<a href='/topic/show?id=0e311010591a' target=_blank style='color:#2F92EE;'>#食管#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=101059, encryptionId=0e311010591a, topicName=食管)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Tue Dec 01 09:35:30 CST 2020, time=2020-12-01, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1473512, encodeId=04cf14e3512b2, content=<a href='/topic/show?id=4fd1e102257' target=_blank style='color:#2F92EE;'>#病理学#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71022, encryptionId=4fd1e102257, topicName=病理学)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c8ce7226273, createdName=jktdtl, createdTime=Tue Dec 01 09:35:30 CST 2020, time=2020-12-01, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=903498, encodeId=69379034984f, content=我也是第一次听说这个病。, beContent=null, objectType=article, channel=null, level=null, likeNumber=80, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201120/5008534db22c474a844fbd107ff83af2/d3e20fc133b542208ff15e7f2b575329.jpg, createdBy=c7015311102, createdName=Jessie Zhang, createdTime=Mon Nov 30 10:24:18 CST 2020, time=2020-11-30, status=1, ipAttribution=)]
    2020-11-30 Jessie Zhang

    我也是第一次听说这个病。

    0

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患者,女性,31岁,2岁检查血性腹泻时被诊断为克罗恩病,因为反复性吞咽困难就诊。患者克罗恩病进展导致其在10岁时接受了全结肠切除术及末端回肠造口术,之后一直未接受治疗。直到24岁时,由于患者邻近造口术的末端回肠部位出现了明显的造口周瘘管才开始治疗。使用硫唑嘌呤和英夫利西单抗诱导治疗后瘘管闭合。由于个人原因,患者在开始服药12个月后停用了这两种药物。