Neurology:脊髓空洞症的罕见病因

2018-08-23 杨中华 脑血管病及重症文献导读

60岁,男性。进展性下肢无力、麻木、尿潴留、长期便秘2年,伴发作性严重下肢瘫痪。

60岁,男性。

进展性下肢无力、麻木、尿潴留、长期便秘2年,伴发作性严重下肢瘫痪。

MRI显示颈胸脊髓空洞,和流空信号(下图)。A图,矢状位MRI显示从胸髓到圆锥T2高信号,部分与脊髓的增粗和水肿有关;另外,可见脊髓空洞(上面的两个箭头),颈髓下部向下到T8水平中央管扩张,从T8向下中央管轻度扩张。从T6到圆锥脊髓硬膜背部可见大量匐行流空影。B图,轴位T2显示T4水平脊髓空洞:



C图,矢状位T2显示从C2-3到胸髓可见脊髓空洞,在C7水平脊髓空洞最宽。小脑扁桃体位置正常。D图,早期血管造影显示迂曲的引流静脉(箭头)。E图,后期血管造影显示硬脊膜动静脉瘘(红色箭头),供血动脉为T6肋间动脉,白色箭头为迂曲的引流静脉:



F图和G图,分别为外科结扎术后矢状位和轴位T2显示脊髓空洞消失:



手术后患者临床症状戏剧性改善,并且空洞消失。Finsterer J等也报道过硬脊膜动静脉瘘相关脊髓空洞的病例,预后相似。

原始出处:Zaed I1, Pinto MV1, Mauermann ML1, et al. Teaching NeuroImages: Spinal cord syrinx secondary to a spinal dural arteriovenous fistula. Neurology. 2018 Jul 17;91(3):e295-e296.

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    2019-04-22 yinhl1978
  2. [GetPortalCommentsPageByObjectIdResponse(id=2000554, encodeId=427e2000554ce, content=<a href='/topic/show?id=d5df12695e6' target=_blank style='color:#2F92EE;'>#Neurol#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=16, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=12695, encryptionId=d5df12695e6, topicName=Neurol)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=8217292, createdName=yinhl1978, createdTime=Mon Apr 22 18:02:00 CST 2019, time=2019-04-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1467349, encodeId=de38146e349e3, content=<a href='/topic/show?id=ef32e9539e8' target=_blank style='color:#2F92EE;'>#罕见#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=18, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79539, encryptionId=ef32e9539e8, topicName=罕见)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=29f36868190, createdName=syscxl, createdTime=Sat Aug 25 01:02:00 CST 2018, time=2018-08-25, status=1, ipAttribution=)]
    2018-08-25 syscxl