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 HighWire Citing Articles via Google Scholar Citing Articles via Scopus Google Scholar Articles by Comi, A. M. Search for Related Content PubMed PubMed Citation Articles by Comi, A. M. Social Bookmarking                         What's this?

Topical Review: Pathophysiology of Sturge-Weber Syndrome

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

Sturge-Weber syndrome is a neurocutaneous disorder classically presenting with a facial port-wine stain, vascular eye abnormalities, and an ipsilateral occipital leptomeningeal angioma. Children with Sturge-Weber syndrome often develop progressive neurologic problems. Data on the pathophysiology of Sturge-Weber syndrome are briefly reviewed. The embryologic, genetic, and pathologic considerations are discussed, as are theories regarding the mechanisms of the degenerative brain changes. Sturge-Weber syndrome likely results from an early embryologic malformation of vascular development affecting the development of the nearby skin, eye, and brain structures. Studies suggest that complex molecular interactions contribute to the abnormal development and function of blood vessels in Sturge-Weber syndrome. Neurologic deterioration in Sturge-Weber syndrome is likely secondary to impaired blood flow to the brain and is worsened by the presence of seizures. Insights from related areas are discussed, and future research studies are suggested. (J Child Neurol 2003;18:509—516).

Journal of Child Neurology, Vol. 18, No. 8, 509-516 (2003)
DOI: 10.1177/08830738030180080701


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

This article has been cited by other articles:

				J. Reesman, R. Gray, S. J. Suskauer, L. M. Ferenc, E. H. Kossoff, D. D. M. Lin, E. Turin, A. M. Comi, P. J. Brice, and T. A. Zabel Hemiparesis Is a Clinical Correlate of General Adaptive Dysfunction in Children and Adolescents with Sturge-Weber Syndrome J Child Neurol, June 1, 2009; 24(6): 701 - 708. [Abstract] [PDF]

				M.E. Adams, S.E. Aylett, W. Squier, and W. Chong A Spectrum of Unusual Neuroimaging Findings in Patients with Suspected Sturge-Weber Syndrome AJNR Am. J. Neuroradiol., February 1, 2009; 30(2): 276 - 281. [Abstract] [Full Text] [PDF]

				E. H. Kossoff, M. Balasta, L. M. Hatfield, C. U. Lehmann, and A. M. Comi Self-Reported Treatment Patterns in Patients With Sturge-Weber Syndrome and Migraines J Child Neurol, June 1, 2007; 22(6): 720 - 726. [Abstract] [PDF]

				D.D.M. Lin, P. Gailloud, E.F. McCarthy, and A.M. Comi Oromaxillofacial osseous abnormality in sturge-weber syndrome: case report and review of the literature. AJNR Am. J. Neuroradiol., February 1, 2006; 27(2): 274 - 277. [Abstract] [Full Text] [PDF]

				A. V. Yallapragada, J. K. Cure, and K. R. Holden Sturge-Weber Syndrome Variant With Atypical Intracranial Findings: Case Report J Child Neurol, February 1, 2006; 21(2): 155 - 157. [Abstract] [PDF]