Movement disorders:肝豆状核变性,大脑结构如何影响认知?

2022-06-25 Freeman MedSci原创

患者的细微认知障碍代表了一种与弥漫性皮质和白质病变相关的独特的神经系统表型

威尔逊病(WD)是一种常染色体隐性铜代谢紊乱,表现为运动障碍、精神特征或肝病。 认知障碍在有神经系统表现的病人中也很常见,但通常是轻微的。 各种领域,包括抽象推理、执行功能、记忆、处理速度、视觉空间功能和社会认知都可能受到影响。

图1: 论文封面图


在没有脑病的肝病患者中也有亚临床功能障碍的报道。 了解认知症状的解剖学基础可能为了解WD中神经系统受累的演变提供启示。

只有少数研究对WD的认知障碍和神经影像学异常之间的关系进行了研究。 Seniow等人发现,基底神经节局部T2加权信号异常的患者与那些有更多弥漫性异常的患者的认知能力相似。 他们得出结论,基底神经节功能障碍是WD认知障碍的主要原因。

Frota等人证明,在多个领域有缺陷的患者在半定量磁共振成像(MRI)量表上的得分更高。 Dong等人利用定量分析报告说,对前瞻性记忆的测量与特定白质束的弥散异常和右眶额回的皮质厚度相关。 基底神经节内部和外部的病变在多大程度上有助于WD认知功能的其他方面仍不清楚。

藉此,英国UCL Queen Square Institute of Neurology的Samuel Shribman等人,在40名前瞻性招募的WD患者中使用了T1加权流体增强反转恢复(FLAIR)、扩散加权成像(DWI)和易感性加权成像(SWI)序列的定量、全脑分析,以确定神经系统受累的成像相关因素和铜指数。

 

图2:论文结果图


他们发现:神经系统表现的患者的抽象推理、执行功能、处理速度、计算和视觉空间功能得分低于肝脏表现的患者,并与神经系统检查的分值相关。

即使在控制了神经系统的严重程度后,抽象推理和语音流畅性的缺陷也与较低的putamen容积有关。

大约一半的肝病患者在面孔记忆、认知灵活性或联想学习方面的表现比正常数据差。这些缺陷与广泛的皮质萎缩和/或白质弥散异常有关。

该研究的重要意义在于发现了:患者的细微认知障碍代表了一种与弥漫性皮质和白质病变相关的独特的神经系统表型。这可能先于以运动障碍和执行功能障碍为特征的经典神经系统表型,并与基底节损伤有关。WD的二元表型分类可能不再合适。



原文出处:
Shribman S, Burrows M, Convery R, et al. Neuroimaging Correlates of Cognitive Deficits in Wilson’s Disease. _Movement Disorders_. Published online June 20, 2022:mds.29123. doi:[10.1002/mds.29123](https://doi.org/10.1002/mds.29123)

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    2023-02-14 cmsvly
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    2023-06-03 anminleiryan
  5. [GetPortalCommentsPageByObjectIdResponse(id=1738136, encodeId=52f51e3813606, content=<a href='/topic/show?id=e10b600329' target=_blank style='color:#2F92EE;'>#disorders#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6003, encryptionId=e10b600329, topicName=disorders)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=efbf34357637, createdName=ms2186806800017884, createdTime=Tue Aug 23 09:35:38 CST 2022, time=2022-08-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1784520, encodeId=8cbb1e845201e, content=<a href='/topic/show?id=a727600194' target=_blank style='color:#2F92EE;'>#Disord#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=41, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6001, encryptionId=a727600194, topicName=Disord)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=aad0444, createdName=cmsvly, createdTime=Tue Feb 14 00:35:38 CST 2023, time=2023-02-14, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2077704, encodeId=059620e7704f3, content=<a href='/topic/show?id=67cc600242' target=_blank style='color:#2F92EE;'>#disorder#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=34, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6002, encryptionId=67cc600242, topicName=disorder)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=3fc52500170, createdName=12498cbcm00(暂无昵称), createdTime=Fri Jul 22 03:35:38 CST 2022, time=2022-07-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1878546, encodeId=fb3c18e854697, content=<a href='/topic/show?id=c157696175' target=_blank style='color:#2F92EE;'>#ERS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6961, encryptionId=c157696175, topicName=ERS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e93e105, createdName=anminleiryan, createdTime=Sat Jun 03 08:35:38 CST 2023, time=2023-06-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2013910, encodeId=278e20139100b, content=<a href='/topic/show?id=7d69598468' target=_blank style='color:#2F92EE;'>#Dis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=28, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=5984, encryptionId=7d69598468, topicName=Dis)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c689267, createdName=cmj8wellington, createdTime=Sun Jul 17 10:35:38 CST 2022, time=2022-07-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1424049, encodeId=0c84142404948, content=<a href='/topic/show?id=34db439533f' target=_blank style='color:#2F92EE;'>#大脑结构#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=43953, encryptionId=34db439533f, topicName=大脑结构)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=3d9a3889985, createdName=shanyongle, createdTime=Sat Jun 25 03:35:38 CST 2022, time=2022-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1463817, encodeId=ad18146381ead, content=<a href='/topic/show?id=7d27381288e' target=_blank style='color:#2F92EE;'>#变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=38128, encryptionId=7d27381288e, topicName=变性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f69f6545041, createdName=huangdf, createdTime=Sat Jun 25 03:35:38 CST 2022, time=2022-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1556547, encodeId=b671155654eed, content=<a href='/topic/show?id=ab7e85001cf' target=_blank style='color:#2F92EE;'>#脑结构#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=34, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=85001, encryptionId=ab7e85001cf, topicName=脑结构)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=4f3114785325, createdName=wmr116, createdTime=Sat Jun 25 03:35:38 CST 2022, time=2022-06-25, status=1, ipAttribution=)]
  6. [GetPortalCommentsPageByObjectIdResponse(id=1738136, encodeId=52f51e3813606, content=<a href='/topic/show?id=e10b600329' target=_blank style='color:#2F92EE;'>#disorders#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6003, encryptionId=e10b600329, topicName=disorders)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=efbf34357637, createdName=ms2186806800017884, createdTime=Tue Aug 23 09:35:38 CST 2022, time=2022-08-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1784520, encodeId=8cbb1e845201e, content=<a href='/topic/show?id=a727600194' target=_blank style='color:#2F92EE;'>#Disord#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=41, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6001, encryptionId=a727600194, topicName=Disord)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=aad0444, createdName=cmsvly, createdTime=Tue Feb 14 00:35:38 CST 2023, time=2023-02-14, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2077704, encodeId=059620e7704f3, content=<a href='/topic/show?id=67cc600242' target=_blank style='color:#2F92EE;'>#disorder#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=34, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6002, encryptionId=67cc600242, topicName=disorder)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=3fc52500170, createdName=12498cbcm00(暂无昵称), createdTime=Fri Jul 22 03:35:38 CST 2022, time=2022-07-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1878546, encodeId=fb3c18e854697, content=<a href='/topic/show?id=c157696175' target=_blank style='color:#2F92EE;'>#ERS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6961, encryptionId=c157696175, topicName=ERS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e93e105, createdName=anminleiryan, createdTime=Sat Jun 03 08:35:38 CST 2023, time=2023-06-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2013910, encodeId=278e20139100b, content=<a href='/topic/show?id=7d69598468' target=_blank style='color:#2F92EE;'>#Dis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=28, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=5984, encryptionId=7d69598468, topicName=Dis)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c689267, createdName=cmj8wellington, createdTime=Sun Jul 17 10:35:38 CST 2022, time=2022-07-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1424049, encodeId=0c84142404948, content=<a href='/topic/show?id=34db439533f' target=_blank style='color:#2F92EE;'>#大脑结构#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=43953, encryptionId=34db439533f, topicName=大脑结构)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=3d9a3889985, createdName=shanyongle, createdTime=Sat Jun 25 03:35:38 CST 2022, time=2022-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1463817, encodeId=ad18146381ead, content=<a href='/topic/show?id=7d27381288e' target=_blank style='color:#2F92EE;'>#变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=38128, encryptionId=7d27381288e, topicName=变性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f69f6545041, createdName=huangdf, createdTime=Sat Jun 25 03:35:38 CST 2022, time=2022-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1556547, encodeId=b671155654eed, content=<a href='/topic/show?id=ab7e85001cf' target=_blank style='color:#2F92EE;'>#脑结构#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=34, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=85001, encryptionId=ab7e85001cf, topicName=脑结构)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=4f3114785325, createdName=wmr116, createdTime=Sat Jun 25 03:35:38 CST 2022, time=2022-06-25, status=1, ipAttribution=)]
  7. [GetPortalCommentsPageByObjectIdResponse(id=1738136, encodeId=52f51e3813606, content=<a href='/topic/show?id=e10b600329' target=_blank style='color:#2F92EE;'>#disorders#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6003, encryptionId=e10b600329, topicName=disorders)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=efbf34357637, createdName=ms2186806800017884, createdTime=Tue Aug 23 09:35:38 CST 2022, time=2022-08-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1784520, encodeId=8cbb1e845201e, content=<a href='/topic/show?id=a727600194' target=_blank style='color:#2F92EE;'>#Disord#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=41, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6001, encryptionId=a727600194, topicName=Disord)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=aad0444, createdName=cmsvly, createdTime=Tue Feb 14 00:35:38 CST 2023, time=2023-02-14, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2077704, encodeId=059620e7704f3, content=<a href='/topic/show?id=67cc600242' target=_blank style='color:#2F92EE;'>#disorder#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=34, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6002, encryptionId=67cc600242, topicName=disorder)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=3fc52500170, createdName=12498cbcm00(暂无昵称), createdTime=Fri Jul 22 03:35:38 CST 2022, time=2022-07-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1878546, encodeId=fb3c18e854697, content=<a href='/topic/show?id=c157696175' target=_blank style='color:#2F92EE;'>#ERS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6961, encryptionId=c157696175, topicName=ERS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e93e105, createdName=anminleiryan, createdTime=Sat Jun 03 08:35:38 CST 2023, time=2023-06-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2013910, encodeId=278e20139100b, content=<a href='/topic/show?id=7d69598468' target=_blank style='color:#2F92EE;'>#Dis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=28, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=5984, encryptionId=7d69598468, topicName=Dis)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c689267, createdName=cmj8wellington, createdTime=Sun Jul 17 10:35:38 CST 2022, time=2022-07-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1424049, encodeId=0c84142404948, content=<a href='/topic/show?id=34db439533f' target=_blank style='color:#2F92EE;'>#大脑结构#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=43953, encryptionId=34db439533f, topicName=大脑结构)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=3d9a3889985, createdName=shanyongle, createdTime=Sat Jun 25 03:35:38 CST 2022, time=2022-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1463817, encodeId=ad18146381ead, content=<a href='/topic/show?id=7d27381288e' target=_blank style='color:#2F92EE;'>#变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=38128, encryptionId=7d27381288e, topicName=变性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f69f6545041, createdName=huangdf, createdTime=Sat Jun 25 03:35:38 CST 2022, time=2022-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1556547, encodeId=b671155654eed, content=<a href='/topic/show?id=ab7e85001cf' target=_blank style='color:#2F92EE;'>#脑结构#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=34, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=85001, encryptionId=ab7e85001cf, topicName=脑结构)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=4f3114785325, createdName=wmr116, createdTime=Sat Jun 25 03:35:38 CST 2022, time=2022-06-25, status=1, ipAttribution=)]
    2022-06-25 huangdf
  8. [GetPortalCommentsPageByObjectIdResponse(id=1738136, encodeId=52f51e3813606, content=<a href='/topic/show?id=e10b600329' target=_blank style='color:#2F92EE;'>#disorders#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6003, encryptionId=e10b600329, topicName=disorders)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=efbf34357637, createdName=ms2186806800017884, createdTime=Tue Aug 23 09:35:38 CST 2022, time=2022-08-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1784520, encodeId=8cbb1e845201e, content=<a href='/topic/show?id=a727600194' target=_blank style='color:#2F92EE;'>#Disord#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=41, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6001, encryptionId=a727600194, topicName=Disord)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=aad0444, createdName=cmsvly, createdTime=Tue Feb 14 00:35:38 CST 2023, time=2023-02-14, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2077704, encodeId=059620e7704f3, content=<a href='/topic/show?id=67cc600242' target=_blank style='color:#2F92EE;'>#disorder#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=34, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6002, encryptionId=67cc600242, topicName=disorder)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=3fc52500170, createdName=12498cbcm00(暂无昵称), createdTime=Fri Jul 22 03:35:38 CST 2022, time=2022-07-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1878546, encodeId=fb3c18e854697, content=<a href='/topic/show?id=c157696175' target=_blank style='color:#2F92EE;'>#ERS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6961, encryptionId=c157696175, topicName=ERS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e93e105, createdName=anminleiryan, createdTime=Sat Jun 03 08:35:38 CST 2023, time=2023-06-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2013910, encodeId=278e20139100b, content=<a href='/topic/show?id=7d69598468' target=_blank style='color:#2F92EE;'>#Dis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=28, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=5984, encryptionId=7d69598468, topicName=Dis)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c689267, createdName=cmj8wellington, createdTime=Sun Jul 17 10:35:38 CST 2022, time=2022-07-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1424049, encodeId=0c84142404948, content=<a href='/topic/show?id=34db439533f' target=_blank style='color:#2F92EE;'>#大脑结构#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=43953, encryptionId=34db439533f, topicName=大脑结构)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=3d9a3889985, createdName=shanyongle, createdTime=Sat Jun 25 03:35:38 CST 2022, time=2022-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1463817, encodeId=ad18146381ead, content=<a href='/topic/show?id=7d27381288e' target=_blank style='color:#2F92EE;'>#变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=38128, encryptionId=7d27381288e, topicName=变性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f69f6545041, createdName=huangdf, createdTime=Sat Jun 25 03:35:38 CST 2022, time=2022-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1556547, encodeId=b671155654eed, content=<a href='/topic/show?id=ab7e85001cf' target=_blank style='color:#2F92EE;'>#脑结构#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=34, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=85001, encryptionId=ab7e85001cf, topicName=脑结构)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=4f3114785325, createdName=wmr116, createdTime=Sat Jun 25 03:35:38 CST 2022, time=2022-06-25, status=1, ipAttribution=)]
    2022-06-25 wmr116

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肝豆状核变性:AASLD多学科诊疗实践|指南解读

肝豆状核变性又称威尔逊病,是一种先天性铜代谢障碍所致的以肝硬化和脑部病变为特征的遗传性疾病。

​肝豆状核变性:中西医结合治疗|疑难探究

肝豆状核变性,也称为Wilson病(WD)是一种潜在可治疗的遗传性铜代谢障碍,其特征是铜的病理性积累。

王共强:肝豆状核变性螯合治疗与铜缺乏

铜是一种必需的微量金属,是催化几种重要的细胞酶所必需的。然而,由于过量的铜可能催化有毒活性氧物质的产生,因此铜的转运和细胞铜含量受到严格的控制。

中华医学会第一届全国罕见病学术年会(CSRD 2023):肝豆状核变性基因筛查临床应用的前期研究

本次大会,来自复旦大学附属儿科医院的唐美玲教授为我们分享《肝豆状核变性基因筛查临床应用的前期研究》议题。梅斯医学整理重点分享给各位。

Front Neurol:揭示Wilson病认知功能对步态的影响

该研究通过探索认知障碍对WD患者步态的影响及其神经机制,将为临床通过认知和运动双重任务训练方法改善步态障碍提供理论依据。

肝豆状核变性:Leipzig评分诊断系统

肝豆状核变性是一种常染色体隐性遗传病,其病变基础为编码铜跨膜转运蛋白的基因ATP7B发生突变,导致铜在肝脏、大脑和其他组织器官中过度沉积,从而引起各种临床表现,其中最常见的是进行性肝病和神经精神障碍。

《2022年美国肝病学会实践指导:肝豆状核变性的诊断和治疗》意见要点

首都医科大学附属北京友谊医院肝病中心 · 2022-12-20

2022 BASL实践指南:Wilson's病(肝豆状核变性)的调查和管理

英国肝脏研究协会(BASL,British Association for Studies of the Liver) · 2022-06-07

中国肝豆状核变性诊治指南2021

中华医学会神经病学分会 · 2021-04-09

2012欧洲肝脏研究会肝豆状核变性(Wilson病)的诊治指南

欧洲肝脏研究学会(EASL,European Association for the Study of the Liver) · 2016-06-08