Neurology:可治的罕见病因所致舞蹈症

2018-10-12 zyx整理 神经科病例撷英拾粹

72岁女性,出现进行性舞蹈样动作,累及右侧半身和口面部肌肉(视频)。余查体无殊,没有共济失调。

72岁女性,出现进行性舞蹈样动作,累及右侧半身和口面部肌肉(视频)。余查体无殊,没有共济失调。脑MRI,血糖,甲状腺功能和风湿系列检查均未见明显异常。亨廷顿病和C9orf72扩增的基因检测结果为阴性。开始的红细胞比容和血红蛋白水平正常,但在舞蹈症出现1年后异常升高(56%和18.5g/dL)。JAK2V617F突变呈阳性,提示真性红细胞增多症所致舞蹈病。放血和羟基脲治疗后舞蹈症消失。神经科医生遇到老年舞蹈症患者时,应考虑到这种罕见的但可治疗的疾病实体,即使最初的红细胞比容值正常。


(真性红细胞增多症患者主要表现为右偏侧舞蹈症和口面部肌肉轻微受累,在放血和羟基脲治疗后完全好转)

原始出处:Rossi M1, Cammarota A2, Merello M2, Nogues M2. Teaching Video NeuroImages: A treatable rare cause of chorea. Neurology. 2018 Sep 11;91(11):e1089. 

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    2019-05-13 yinhl1978
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    2018-10-13 syscxl
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topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Fri Oct 12 07:27:37 CST 2018, time=2018-10-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=348991, encodeId=7cdb34899193, content=学习了长知识, beContent=null, objectType=article, channel=null, level=null, likeNumber=40, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Fri Oct 12 07:27:33 CST 2018, time=2018-10-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=348990, encodeId=5aaa34899076, content=学习了长知识, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Fri Oct 12 07:27:28 CST 2018, time=2018-10-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=348989, encodeId=5dce348989aa, content=学习了长知识, beContent=null, objectType=article, channel=null, level=null, likeNumber=18, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Fri Oct 12 07:27:23 CST 2018, time=2018-10-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=348988, encodeId=eca43489884f, content=学习了长知识, beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Fri Oct 12 07:27:18 CST 2018, time=2018-10-12, status=1, ipAttribution=)]
    2018-10-12 飛歌

    学习了很有用不错

    0

  5. 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channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Fri Oct 12 07:27:18 CST 2018, time=2018-10-12, status=1, ipAttribution=)]
    2018-10-12 Y—xianghai

    学习了长知识

    0

  6. 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replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Fri Oct 12 07:27:28 CST 2018, time=2018-10-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=348989, encodeId=5dce348989aa, content=学习了长知识, beContent=null, objectType=article, channel=null, level=null, likeNumber=18, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Fri Oct 12 07:27:23 CST 2018, time=2018-10-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=348988, encodeId=eca43489884f, content=学习了长知识, beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Fri Oct 12 07:27:18 CST 2018, time=2018-10-12, status=1, ipAttribution=)]
    2018-10-12 Y—xianghai

    学习了长知识

    0

  7. 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channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Fri Oct 12 07:27:18 CST 2018, time=2018-10-12, status=1, ipAttribution=)]
    2018-10-12 Y—xianghai

    学习了长知识

    0

  8. 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channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Fri Oct 12 07:27:18 CST 2018, time=2018-10-12, status=1, ipAttribution=)]
    2018-10-12 Y—xianghai

    学习了长知识

    0

  9. 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channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Fri Oct 12 07:27:18 CST 2018, time=2018-10-12, status=1, ipAttribution=)]
    2018-10-12 Y—xianghai

    学习了长知识

    0

  10. 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channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20201006/4db877c2887649beb09cb5effd0b38c7/916f66edd48a4e77b20ebc3e14b6e45c.jpg, createdBy=141e1980133, createdName=Y—xianghai, createdTime=Fri Oct 12 07:27:18 CST 2018, time=2018-10-12, status=1, ipAttribution=)]
    2018-10-12 Y—xianghai

    学习了长知识

    0

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