Matsanga Leyila Kaseka1, Jonathan Yehouda Bitton2, Jean-Claude Décarie3, Philippe Major2. 1. Division of Neurology, Department of Neurosciences and Pediatrics, CHU Sainte Justine, University of Montreal, Montreal, Canada. Electronic address: lkaseka@gmail.com. 2. Division of Neurology, Department of Neurosciences and Pediatrics, CHU Sainte Justine, University of Montreal, Montreal, Canada. 3. Department of Radiology, CHU Sainte Justine, University of Montreal, Montreal, Canada.
Abstract
BACKGROUND: Sturge-Weber syndrome is characterized by a facial port-wine stain associated with either or both a retinal angioma and a cerebral pial angioma. Because a pial angioma may not be evident on the initial imaging studies, individuals at risk for epilepsy are often not identified before their first seizure. The aim of this study is to identify predictive factors predisposing Sturge-Weber patients to epilepsy. METHODS: The medical archives and photography database of our institution were reviewed to identify Sturge-Weber Syndrome patients followed up between 1990 and 2015. Patients without epilepsy were compared with patients with epilepsy based on the location of the port-wine stain, its extent and cerebral imaging. RESULTS: Twenty-four patients were included in the study. Thirteen did not develop epilepsy. Patients with bilateral port-wine stain were at higher risk of epilepsy (P = 0.03). Unilateral port-wine stain did not increase the risk of epilepsy (P = 0.29) regardless of its extent. The presence of developmental venous anomalies on brain imaging was also associated with a higher risk of epilepsy (P = 0.03). CONCLUSIONS: Bilateral facial port-wine stain and cerebral developmental venous anomalies increase the risk of epilepsy in Sturge-Weber syndrome patients. Because they can be detected at birth, they might guide preventive management and follow-up.
BACKGROUND:Sturge-Weber syndrome is characterized by a facial port-wine stain associated with either or both a retinal angioma and a cerebral pial angioma. Because a pial angioma may not be evident on the initial imaging studies, individuals at risk for epilepsy are often not identified before their first seizure. The aim of this study is to identify predictive factors predisposing Sturge-Weber patients to epilepsy. METHODS: The medical archives and photography database of our institution were reviewed to identify Sturge-Weber Syndromepatients followed up between 1990 and 2015. Patients without epilepsy were compared with patients with epilepsy based on the location of the port-wine stain, its extent and cerebral imaging. RESULTS: Twenty-four patients were included in the study. Thirteen did not develop epilepsy. Patients with bilateral port-wine stain were at higher risk of epilepsy (P = 0.03). Unilateral port-wine stain did not increase the risk of epilepsy (P = 0.29) regardless of its extent. The presence of developmental venous anomalies on brain imaging was also associated with a higher risk of epilepsy (P = 0.03). CONCLUSIONS: Bilateral facial port-wine stain and cerebral developmental venous anomalies increase the risk of epilepsy in Sturge-Weber syndromepatients. Because they can be detected at birth, they might guide preventive management and follow-up.
Authors: Lindsay F Smegal; Alison J Sebold; Adrienne M Hammill; Csaba Juhász; Warren D Lo; Daniel K Miles; Angus A Wilfong; Alex V Levin; Brian Fisher; Karen L Ball; Anna L Pinto; Anne M Comi Journal: Pediatr Neurol Date: 2021-03-05 Impact factor: 4.210
Authors: Sara Sabeti; Karen L Ball; Craig Burkhart; Lawrence Eichenfield; Esteban Fernandez Faith; Ilona J Frieden; Roy Geronemus; Deepti Gupta; Andrew C Krakowski; Moise L Levy; Denise Metry; J Stuart Nelson; Megha M Tollefson; Kristen M Kelly Journal: JAMA Dermatol Date: 2021-01-01 Impact factor: 10.282