Document Detail


Medically intractable epilepsy in Sturge-Weber syndrome is associated with cortical malformation: implications for surgical therapy.
MedLine Citation:
PMID:  19780796     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
PURPOSE: Anecdotal reports have described cortical malformations in epileptic patients with Sturge-Weber syndrome (SWS). No data are available regarding the prevalence and significance of this association. METHODS: We reviewed retrospectively the clinical profile, preoperative magnetic resonance imaging (MRI) studies, and pathology reports of all patients with SWS and medically intractable epilepsy evaluated in our epilepsy surgery program between 1979 and 2006. RESULTS: Twelve patients (male/female = 7/5) were identified. Mean age at seizure onset was 11.1 +/- 16.7 months. Seizures occurred daily in seven patients and weekly in five patients. A facial port-wine stain was noted in 10 cases. Eleven patients evidenced developmental delay and eight were hemiparetic. Eight patients underwent excisional surgery for epilepsy (mean age 10.3 +/- 6.5 year), including hemispherectomy (n = 4) and focal cortical resection (n = 4). Tissue was available for neuropathology in six operated cases and revealed polymicrogyria (n = 3) and cortical dysplasia (n = 4). Polymicrogyria was associated with cortical dysplasia in one child. Brain MRIs were reviewed in 10 of 12 patients and were consistent with cortical malformations in all cases. CONCLUSIONS: We conclude that cortical malformations are frequent in patients with medically intractable epilepsy and Sturge-Weber-syndrome and may be the primary cause of epilepsy.
Authors:
Bruno Maton; Pavel Krsek; Prasanna Jayakar; Trevor Resnick; Monica Koehn; Glenn Morrison; John Ragheb; Amilcar Castellano-Sanchez; Michael Duchowny
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't     Date:  2009-09-22
Journal Detail:
Title:  Epilepsia     Volume:  51     ISSN:  1528-1167     ISO Abbreviation:  Epilepsia     Publication Date:  2010 Feb 
Date Detail:
Created Date:  2010-03-24     Completed Date:  2010-04-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  2983306R     Medline TA:  Epilepsia     Country:  United States    
Other Details:
Languages:  eng     Pagination:  257-67     Citation Subset:  IM    
Affiliation:
Miami Children's Hospital, The Brain Institute, Miami, Florida 33155-4079, USA.
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MeSH Terms
Descriptor/Qualifier:
Brain Mapping
Cerebral Cortex / abnormalities*,  surgery
Child, Preschool
Electroencephalography
Epilepsy / diagnosis*,  epidemiology,  surgery*
Female
Functional Laterality / physiology
Hemispherectomy
Humans
Infant
Infant, Newborn
Magnetic Resonance Imaging
Male
Malformations of Cortical Development / diagnosis,  surgery
Neuropsychological Tests
Preoperative Care
Prognosis
Sturge-Weber Syndrome / diagnosis*,  epidemiology,  surgery*
Tomography, X-Ray Computed

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