Intracranial Artery Dissection in an Adolescent With Marfan Syndrome

• Published in: Pediatric neurology Vol. 45; no. 1; pp. 39 - 41
• Main Authors: Maski, Kiran P., MD, Sengupta, Soma, MD, Silvera, Michelle, MD, Rivkin, Michael J., MD
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01-07-2011; Elsevier
• Subjects: Aneurysm, Dissecting - complications; Aneurysm, Dissecting - diagnosis; Biological and medical sciences; Humans; Intracranial Aneurysm - complications; Intracranial Aneurysm - diagnosis; Magnetic Resonance Angiography; Male; Marfan Syndrome - complications; Marfan Syndrome - diagnosis; Medical sciences; Neurology; Pediatrics; Tomography, X-Ray Computed; Vascular diseases and vascular malformations of the nervous system; Young Adult; Human; Connective tissue disease; Intracranial; Skin disease; Nervous system diseases; Elastic tissue disease; Cardiovascular disease; Marfan syndrome; Genetic disease; Artery dissection; Arterial disease; Vascular disease; Dissecting aneurysm; Systemic disease; Adolescent
• ISSN: 0887-8994; 1873-5150
• DOI: 10.1016/j.pediatrneurol.2010.12.011

Abstract 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.