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Marfan 综合征相关的椎基底动脉延长扩张症。

Vertebrobasilar dolichoectasia associated with Marfan syndrome.

机构信息

Cerebrovascular Program and Division of Neuro-Critical Care, Department of Neurology, and the Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD; and the Department of Neurology, Emory University School of Medicine, Atlanta, GA.

出版信息

J Stroke Cerebrovasc Dis. 2000 Jul-Aug;9(4):196-8. doi: 10.1053/jscd.2000.7224.

Abstract

We report a 37-year-old woman with Marfan syndrome (MFS) who presented with acute myelopathy secondary to a subdural spinal hematoma. The patient died of a subarachnoid hemorrhage 4 days later. Autopsy showed a markedly ectatic vertebrobasilar system with fragmentation of the internal elastic lamina. Microscopic examination of the aorta similarly showed a fragmented internal elastic lamina. We discuss the implications of our patient's early onset vertebrobasilar dolichoectasia; this intracranial disease represents a rare cause of subarachnoid hemorrhage in MFS.

摘要

我们报告了一例 37 岁的马凡综合征(MFS)患者,其因硬脑膜下脊髓血肿导致急性脊髓病。4 天后,患者死于蛛网膜下腔出血。尸检显示椎基底动脉系统明显扩张,内弹力膜断裂。主动脉的显微镜检查同样显示内弹力膜断裂。我们讨论了我们患者早发的椎基底动脉梭形扩张的意义;这种颅内疾病是 MFS 蛛网膜下腔出血的罕见原因。

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