Intracranial artery dissection in an adolescent with Marfan syndrome.

Pediatr Neurol

Department of Neurology, Children's Hospital Boston, Boston, Massachusetts 02115, USA.

Published: July 2011

Marfan syndrome is an autosomal dominant connective tissue disorder commonly due to mutation of the fibrillin-1 (FBN-1) gene that causes disruption of elastic fibers in large- and medium-size arteries and predisposes to aneurysm formation and arterial dissection. Cardiovascular complications occur in most patients with Marfan syndrome, but interestingly, neurovascular complications of Marfan syndrome are rare. We present a novel case of an adolescent with Marfan syndrome with spontaneous intracranial cerebral artery dissection and ischemic stroke with hemorrhagic transformation. This case is novel in that it reports spontaneous intracranial dissection in a young patient with Marfan syndrome and highlights the rare intrinsic neurovascular complications that can occur in these patients.

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http://dx.doi.org/10.1016/j.pediatrneurol.2010.12.011DOI Listing

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