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Neurology：见于LGI-1自身免疫性疾病的获得性局灶性神经性肌强直

2018-06-08 zyx整理 神经科病例撷英拾粹

自身免疫病过度兴奋神经退行性疾病
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81岁女性，起初被诊断为皮质基底节变性（认知功能下降和左手特殊姿势）。随后出现低钠血症和双侧面臂肌张力障碍样发作（faciobrachial dystonic seizures，FBDS）。

81岁女性，起初被诊断为皮质基底节变性（认知功能下降和左手特殊姿势）。随后出现低钠血症和双侧面臂肌张力障碍样发作（faciobrachial dystonic seizures，FBDS）。 神经系统查体（图A）可见持续不自主连续的左侧第3和第4指屈肌兴奋伴松弛延后。肌电图显示高频自发放电，包括左侧屈指浅肌部和旋前肌神经性肌强直和快速肌纤维颤搐（图B）。检测到LGI-1免疫球蛋白G（IgG）1，但未发现CASPR2-IgG（图C-E）。肿瘤筛查提示乳腺导管内原位癌。静脉注射免疫球蛋白后脑病和FBDS好转。脑病伴周围神经过度兴奋可貌似神经退行性疾病，鉴别诊断时应考虑到LGI-1/CASPR2自身免疫病的可能。


（图：A：持续性左手第三和第四指屈曲；B：肌电图提示异常的高爆自发放电；C-E：基于细胞的免疫荧光检测证实LGI-1-IgG阳性，而CASPR2-IgG阴性）


（患者的神经系统查体提示受累左手持续性肌肉收缩伴松弛延后，左侧指浅屈肌肌电图可见高频自发放电）

原始出处：López Chiriboga AS, Matsumoto J, Sorenson E, Klein CJ, McKeon A.Teaching Video NeuroImages: Acquired focal neuromyotonia in LGI-1 autoimmunity.Neurology. 2018 May 1;90(18):e1636-e1637. 

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    2018-11-07 hywen7326

    #神经性#

    0

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    2019-05-14 yinhl1978

    #Neurol#

    0

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    2018-10-06 pandamao2010

    #获得性#

    0

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    2019-03-30 cathymary

    #局灶性#

    0

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    2018-06-10 124988c7m100暂无昵称

    #免疫性疾病#

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=2035427, encodeId=3b90203542e03, content=<a href='/topic/show?id=d382e42956d' target=_blank style='color:#2F92EE;'>#神经性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=26, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=74295, encryptionId=d382e42956d, topicName=神经性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=927d310, createdName=hywen7326, createdTime=Wed Nov 07 06:31:00 CST 2018, time=2018-11-07, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2000449, encodeId=aa1720004498d, content=<a href='/topic/show?id=d5df12695e6' target=_blank style='color:#2F92EE;'>#Neurol#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=33, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=12695, encryptionId=d5df12695e6, topicName=Neurol)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=8217292, createdName=yinhl1978, createdTime=Tue May 14 06:31:00 CST 2019, time=2019-05-14, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2042616, encodeId=f39e2042616f3, content=<a href='/topic/show?id=999c88014ea' target=_blank style='color:#2F92EE;'>#获得性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=88014, encryptionId=999c88014ea, topicName=获得性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=d4b7363, createdName=pandamao2010, createdTime=Sat Oct 06 09:31:00 CST 2018, time=2018-10-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2009682, encodeId=3f842009682c4, content=<a href='/topic/show?id=c7804e581bc' target=_blank style='color:#2F92EE;'>#局灶性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=39, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=47581, encryptionId=c7804e581bc, topicName=局灶性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=226a143, createdName=cathymary, createdTime=Sat Mar 30 05:31:00 CST 2019, time=2019-03-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1580146, encodeId=2d9a158014623, content=<a href='/topic/show?id=bd2529020e1' target=_blank style='color:#2F92EE;'>#免疫性疾病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=28, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=29020, encryptionId=bd2529020e1, topicName=免疫性疾病)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=df0516593209, createdName=124988c7m100暂无昵称, createdTime=Sun Jun 10 13:31:00 CST 2018, time=2018-06-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=322649, encodeId=86b4322649b8, content=学习了.谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=62, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=7c592194322, createdName=1209e435m98(暂无昵称), createdTime=Sat Jun 09 04:02:26 CST 2018, time=2018-06-09, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=322627, encodeId=7e2132262e41, content=不错的文章值得拥有., beContent=null, objectType=article, channel=null, level=null, likeNumber=59, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=eff51996281, createdName=1e1b8538m79(暂无匿称), createdTime=Fri Jun 08 23:11:46 CST 2018, time=2018-06-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=322619, encodeId=ec28322619fd, content=谢谢分享学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=66, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Q3auHgzwzM4o2XVpJ6449yqDsjibydI5HnJ8MibGxNn4HZiaZib0bdfibbwoz3KQrIyN9AGxq36ZlZRNicYlPfOOWMtwgsajY6mGNFiaPclFdowjD0/0, createdBy=97bd1338592, createdName=清风拂面, createdTime=Fri Jun 08 22:54:16 CST 2018, time=2018-06-08, status=1, ipAttribution=)]
    2018-06-09 1209e435m98(暂无昵称)

    学习了.谢谢分享

    0

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    2018-06-08 1e1b8538m79(暂无匿称)

    不错的文章值得拥有.

    0

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    2018-06-08 清风拂面

    谢谢分享学习

    0

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