Advanced Search

Journal Navigation

Journal Home

Subscriptions

Archive

Contact Us

Table of Contents

Click here for more information

Click here to sign up for SAGE Journal Email Alerts today!

Sign In to gain access to subscriptions and/or personal tools.
Journal of Child Neurology
This Article
Right arrow Full Text (PDF)
Right arrow References
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Saved Citations
Right arrow Download to citation manager
Right arrowRequest Permissions
Right arrow Request Reprints
Right arrow Add to My Marked Citations
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Right arrow Citing Articles via Scopus
Google Scholar
Right arrow Articles by Sujansky, E.
Right arrow Articles by Conradi, S.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Sujansky, E.
Right arrow Articles by Conradi, S.
Social Bookmarking
Add to CiteULike   Add to Complore   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Reddit   Add to Technorati   Add to Twitter  
What's this?

Sturge-Weber Syndrome: Age of Onset of Seizures and Glaucoma and the Prognosis for Affected Children

Eva Sujansky, MD, FAAP, FACMG

Division of Genetics, University of Colorado School of Medicine/The Children's Hospital, Denver, CO

Susan Conradi, MS

Division of Genetics, University of Colorado School of Medicine/The Children's Hospital, Denver, CO

Data were obtained on 171 individuals with Sturge-Weber syndrome via questionnaire and medical records. The age of the study group ranged from 2 months to 59 years; the median was 8 years. In addition to the facial location of port-wine stains in the areas of the trigeminal dermatomes present in 170 patients, 45% also had extracranial port-wine stains over the torso and/or extremities, and 17% had other vascular or pigmentary lesions. Seizures were present in 80% of all patients (87% of those with bilateral and 71% of those with unilateral port-wine stains); in all but one case, seizures were associated with port-wine stains in V 1 alone or V1 and V2 trigeminal dermatomes location. The age of onset of seizures ranged from birth to 23 years; 75% had onset of seizures before 1 year of age; these children had an 83% incidence of developmental and academic problems. Fifty-eight percent showed early developmental delay and required special education classes. The rate of retardation showed a decreasing tendency with increasing age of onset of seizures; of the children without seizures, only 6% had developmental delay and 11% required special education classes. Glaucoma was present in 48% of patients (67% unilateral and 33% bilateral). Of all patients with glaucoma, 92% had port-wine stains in both V1 and V2 dermatomes and 8% only in V1. The laterality of glaucoma did not correspond to the trigeminal distribution of the port-wine stains in all instances. Glaucoma was diagnosed during the 1st year of life in 61% and by 5 years in 72%; one patient did not have onset of symptoms until 38 years. The results of our data can serve as a guide for estimating relative risk figures for seizures, glaucoma, and mental retardation in children with Sturge-Weber syndrome. (J Child Neurol 1995;10:49-58).

Journal of Child Neurology, Vol. 10, No. 1, 49-58 (1995)
DOI: 10.1177/088307389501000113
Add to CiteULike CiteULike   Add to Complore Complore   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Reddit Reddit   Add to Technorati Technorati   Add to Twitter Twitter    What's this?


This article has been cited by other articles:


Home page
 J Child NeurolHome page
V. Udani, S. Pujar, P. Munot, S. Maheshwari, and N. Mehta
Natural History and Magnetic Resonance Imaging Follow-up in 9 Sturge-Weber Syndrome Patients and Clinical Correlation
J Child Neurol, April 1, 2007; 22(4): 479 - 483.
[Abstract] [PDF]

Home page
 Arch NeurolHome page
A. M. Comi, P. Mehta, L. A. Hatfield, and M. M. Dowling
Sturge-Weber Syndrome Associated With Other Abnormalities: A Medical Record and Literature Review
Arch Neurol, December 1, 2005; 62(12): 1924 - 1927.
[Abstract] [Full Text] [PDF]

Home page
 J Child NeurolHome page
L. A. Hatfield and E. H. Kossoff
Comorbidity of Epilepsy and Headache in Patients With Sturge-Weber Syndrome
J Child Neurol, August 1, 2005; 20(8): 678 - 682.
[Abstract] [PDF]

Home page
 Br J OphthalmolHome page
T Ong, A Chia, and K K Nischal
Latanoprost in port wine stain related paediatric glaucoma
Br J Ophthalmol, September 1, 2003; 87(9): 1091 - 1093.
[Abstract] [Full Text] [PDF]

Home page
 J Child NeurolHome page
A. M. Comi
Topical Review: Pathophysiology of Sturge-Weber Syndrome
J Child Neurol, August 1, 2003; 18(8): 509 - 516.
[Abstract] [PDF]

Home page
 NeurologyHome page
S.J. Schreiber, F. Doepp, A. Bender, K. Schmierer, and J. M. Valdueza
Diffuse cerebral angiomatosis
Neurology, April 8, 2003; 60(7): 1216 - 1218.
[Full Text] [PDF]

Home page
 J Child NeurolHome page
P. Widdess-Walsh and N. R. Friedman
Left-Sided Facial Nevus With Contralateral Leptomeningeal Angiomatosis in a Child With Sturge-Weber Syndrome: Case Report
J Child Neurol, April 1, 2003; 18(4): 304 - 305.
[Abstract] [PDF]

Home page
 NeurologyHome page
E. H. Kossoff, C. Buck, and J. M. Freeman
Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide
Neurology, December 10, 2002; 59(11): 1735 - 1738.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
J. S. Lee, E. Asano, O. Muzik, D. C. Chugani, C. Juhasz, Z. Pfund, S. Philip, M. Behen, and H. T. Chugani
Sturge-Weber syndrome: Correlation between clinical course and FDG PET findings
Neurology, July 24, 2001; 57(2): 189 - 195.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
A. A. Arzimanoglou, F. Andermann, J. Aicardi, C. Sainte-Rose, M.-A. Beaulieu, J.-G. Villemure, A. Olivier, and Th. Rasmussen
Sturge-Weber syndrome: Indications and results of surgery in 20 patients
Neurology, November 28, 2000; 55(10): 1472 - 1479.
[Abstract] [Full Text] [PDF]

Home page
 J Child NeurolHome page
L. Chapieski, A. Friedman, and D. Lachar
Psychological Functioning in Children and Adolescents With Sturge-Weber Syndrome
J Child Neurol, October 1, 2000; 15(10): 660 - 665.
[Abstract] [PDF]

Home page
 J Child NeurolHome page
B. L. Maria, J. A. Neufeld, L. C. Rosainz, K. Ben-David, W. E. Drane, R. G. Quisling, and L. M. Hamed
High Prevalence of Bihemispheric Structural and Functional Defects in Sturge-Weber Syndrome
J Child Neurol, December 1, 1998; 13(12): 595 - 605.
[Abstract] [PDF]

Home page
 J Child NeurolHome page
G. J. Moore, T. L. Slovis, and H. T. Chugani
Proton Magnetic Resonance Spectroscopy in Children With Sturge-Weber Syndrome
J Child Neurol, July 1, 1998; 13(7): 332 - 335.
[Abstract] [PDF]

Home page
 J Child NeurolHome page
R.-A. Muller, H. T. Chugani, O. Muzik, R. D. Rothermel, and P. K. Chakraborty
Language and Motor Functions Activate Calcified Hemisphere in Patients With Sturge-Weber Syndrome: A Positron Emission Tomography Study
J Child Neurol, October 1, 1997; 12(7): 431 - 437.
[Abstract] [PDF]