Related Experiment Videos
Epilepsy surgery in bilateral Sturge-Weber syndrome
Ingrid E B Tuxhorn1, Heinz W Pannek
1Pediatric Epilepsy Surgery Unit, Klinik Mara, Epilepsy Center Bethel, Bielefeld, Germany.
Insights
Severe epilepsy in infants with Sturge-Weber syndrome can be effectively treated with functional hemispherectomy, leading to significant seizure control and improved quality of life.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Epileptology
Background:
- Sturge-Weber syndrome (SWS) is a rare congenital disorder characterized by a facial birthmark and abnormalities in the brain and spinal cord.
- Severe, drug-refractory focal epilepsy is a common and debilitating manifestation of SWS, particularly in infants.
Observation:
- Two infants with SWS and extensive leptomeningeal angiomatosis presented with severe, intractable focal epilepsy.
- Preoperative evaluations including video-electroencephalography and advanced neuroimaging (CT, MRI) identified epileptogenesis originating from a single hemisphere in both cases.
Findings:
- Both infants underwent successful surgical treatment with functional hemispherectomy.
- Long-term follow-up demonstrated excellent seizure control in both patients post-surgery.
Implications:
- Functional hemispherectomy is a viable and effective surgical option for select pediatric epilepsy cases associated with extensive lesions like SWS.
- Epilepsy surgery candidacy in children should be considered broadly, even in the presence of extensive underlying pathology, to improve seizure outcomes and quality of life.
Abstract:
Two infants with severe drug refractory focal epilepsy caused by Sturge-Weber syndrome and extensive cerebral leptomeningeal angiomatosis were referred for preoperative video-electroencephalographic evaluation. Brain imaging with computed tomography and gadolinium-enhanced magnetic resonance imaging demonstrated bilateral disease in both children with a predominance of involvement of one hemisphere. Clinical examination and neurophysiology with ictal video recording demonstrated epileptogenesis from one hemisphere. Successful surgical treatment with functional hemispherectomy was followed by good long-term seizure control in both patients. The dramatic seizure control was accompanied by markedly improved quality of life for the family and children. These cases indicate that the spectrum of children that may benefit from epilepsy surgery should not be viewed too restrictively, and subsets of children with localization related epilepsy caused by extensive lesions may be resective surgical candidates with a good seizure outcome prognosis.