This Article Full Text (PDF) References Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Comi, A. M. Articles by Hess, E. J. Search for Related Content PubMed PubMed Citation Articles by Comi, A. M. Articles by Hess, E. J. Social Bookmarking                   What's this?

Sturge-Weber Syndrome: Altered Blood Vessel Fibronectin Expression and Morphology

Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, acomi{at}jhmi.edu, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD

Catherine J. C. Weisz, MD

Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, acomi{at}jhmi.edu

Bridget H. Highet, MD

Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, acomi{at}jhmi.edu

Richard L. Skolasky, MD

Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, acomi{at}jhmi.edu

Carlos A. Pardo, MD

Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, acomi{at}jhmi.edu, Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD

Ellen J. Hess, MD,PhD

Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD

Sturge-Weber syndrome presents with vascular malformations of the brain, skin, and eye. Fibronectin has potent effects on angiogenesis, vessel remodeling, and vessel innervation density. To determine fibronectin expression in the blood vessels of Sturge-Weber syndrome brain and skin tissue and to quantify the density and circumference of Sturge-Weber syndrome blood vessels by type compared with controls, we performed in situ hybridization for fibronectin messenger ribonucleic acid (RNA) expression on six Sturge-Weber syndrome cortical brain samples, six epilepsy brain samples, skin from two port-wine stain skin lesions, and two normal skin samples from two subjects with Sturge-Weber syndrome. Fibronectin messenger RNA was expressed in blood vessels and endothelial cells in the parenchyma of both Sturge-Weber syndrome and control brain tissues and in skin samples. Fibronectin expression was significantly reduced by 23% in the Sturge-Weber syndrome meningeal vessels compared with the epilepsy controls (P < .01). Fibronectin expression was significantly increased by 19% in the Sturge-Weber syndrome parenchymal vessels compared with the epilepsy controls (P < .05). No difference was found in the expression of fibronectin in port-wine stain skin blood vessels. The density of leptomeningeal blood vessels in the Sturge-Weber syndrome brain tissue samples was 45% greater than in the epilepsy samples (P < .05). Blood vessel circumference was significantly decreased in the Sturge-Weber syndrome meningeal vessels compared with the controls (27%; P < .05). When blood vessels from different brain regions were compared, fibronectin expression was decreased in Sturge-Weber syndrome meningeal vessels and was increased in the parenchymal vessels. Altered blood vessel fibronectin expression in Sturge-Weber syndrome could contribute to abnormal vascular structure and function in this disorder. (J Child Neurol 2005;20:572—577).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?