Neurology:见于菱脑融合的头部刻板性8字摇晃

2018-06-11 zyx整理 神经科病例撷英拾粹

4岁男孩,因导水管狭窄所致先天性脑积水,出生时接受脑室腹腔分流术治疗。 头颅MRI提示患儿小脑蚓部缺如伴小脑半球相连跨过中线,符合菱脑融合(图)。

4岁男孩,因导水管狭窄所致先天性脑积水,出生时接受脑室腹腔分流术治疗。 头颅MRI提示患儿小脑蚓部缺如伴小脑半球相连跨过中线,符合菱脑融合(图)。患儿总体发育轻度延迟。神经系统查体提示无眼球震颤,动眼神经失用,辨距不良或共济失调。自从1岁开始,患者出现刻板的头部运动,符合节律性8字和左右摇晃(视频)。 这种独特的刻板动作可见于85%的菱脑融合患者。其出现提醒临床医生应注意潜在的菱脑融合或其他后颅窝畸形。


(图:中线矢状位T1WI[A]提示小脑半球分叶征和由半球间囊肿所致的胼胝体后部向上伸出[箭头];冠状位[B]和横断面[C]T2WI可见蚓部发育不全,小脑半球融合,叶形线相连跨过中线;横断面T2WI[D]可见锁眼状第四脑室)


(可见头部刻板性8字摇晃)

原始出处:

Accogli A, Srour M.Teaching Video NeuroImages: Figure 8 head-shaking stereotypy in rhombencephalosynapsis.Neurology. 2018 May 15;90(20):e1832-e1833.

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    2019-03-08 yinhl1978
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    2018-06-13 licz0427
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    2018-06-12 changjiu

    学习一下谢谢

    0

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    2018-06-12 1209e435m98(暂无昵称)

    学习了.谢谢分享

    0

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    2018-06-11 changjiu

    学习一下谢谢

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=2000453, encodeId=e398200045350, content=<a href='/topic/show?id=d5df12695e6' target=_blank style='color:#2F92EE;'>#Neurol#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=22, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=12695, encryptionId=d5df12695e6, topicName=Neurol)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=8217292, createdName=yinhl1978, createdTime=Fri Mar 08 08:09:00 CST 2019, time=2019-03-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1525473, encodeId=4ce815254e314, content=<a href='/topic/show?id=08548885835' target=_blank style='color:#2F92EE;'>#融合#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=18, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=88858, encryptionId=08548885835, topicName=融合)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=08a911636812, createdName=licz0427, createdTime=Wed Jun 13 13:09:00 CST 2018, time=2018-06-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=323436, encodeId=b6de323436c3, content=学习一下谢谢, beContent=null, objectType=article, channel=null, level=null, likeNumber=37, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zSn0GqHrsfLGSUSR8zwnWWLT4FMl5XH8S58HuZslVkLsAPbXDXaliagK7TOxQfmngKibG1yrDonepCLDkcxGk8hS/0, createdBy=b8282062892, createdName=changjiu, createdTime=Tue Jun 12 06:00:17 CST 2018, time=2018-06-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=323417, encodeId=ada232341e01, content=学习了.谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=7c592194322, createdName=1209e435m98(暂无昵称), createdTime=Tue Jun 12 00:27:35 CST 2018, time=2018-06-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=323388, encodeId=44a73233880d, content=学习一下谢谢, beContent=null, objectType=article, channel=null, level=null, likeNumber=28, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zSn0GqHrsfLGSUSR8zwnWWLT4FMl5XH8S58HuZslVkLsAPbXDXaliagK7TOxQfmngKibG1yrDonepCLDkcxGk8hS/0, createdBy=b8282062892, createdName=changjiu, createdTime=Mon Jun 11 23:15:44 CST 2018, time=2018-06-11, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=323383, encodeId=fb9932338363, content=学习学习.知之为知之, beContent=null, objectType=article, channel=null, level=null, likeNumber=42, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTKibA62IFMeUJEmSyIe8DvYFeERuOsfrRd8iano7nENqQU1dRR3tqeQOUL3eqgYgEZRT2t2iaEsib0JEw/132, createdBy=66771693911, createdName=易水河, createdTime=Mon Jun 11 23:06:07 CST 2018, time=2018-06-11, status=1, ipAttribution=)]
    2018-06-11 易水河

    学习学习.知之为知之

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