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Vertebrobasilar dolichoectasia associated with Marfan syndrome
I E Silverman1, D M Berman, G L Dike
1Cerebrovascular 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.
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.
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