Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide.
BACKGROUND
Epilepsy affects 80% of patients with Sturge-Weber syndrome; the majority of seizures begin before the age of 1. When seizures are intractable to medications and unihemispheric, hemispherectomy is often advised.


OBJECTIVE
To examine the natural history of patients who underwent hemispherectomy and identify the outcomes in terms of seizure reduction, cognition, and motor deficits.


METHODS
A questionnaire was mailed to the parents of patients identified by the Sturge-Weber Foundation as having had a hemispherectomy between 1979 and 2001. Forty-six percent (32/70) of the parents responded.


RESULTS
The mean age at onset of seizures was 4 months, and the median age at surgery was 1.2 years. Children had failed to respond to 3.7 anticonvulsants prior to surgery and averaged 387 seizures/month. Forty-seven percent had complications (e.g., hemorrhage and hypertension) in the perioperative period; however, 81% are currently seizure-free, with 53% off anticonvulsants. Hemispherectomy type (anatomic versus functional versus hemidecortication) did not influence outcome. Age at onset of seizures did not predict seizure freedom; however, an older age at hemispherectomy was positively correlated. Postoperative hemiparesis was not more severe than before surgery. Cognitive outcome was not related to the age at operation, side of operation, or seizure freedom.


CONCLUSIONS
Children undergoing hemispherectomy presented at a young age and had frequent seizures for approximately 1 year but are now mostly seizure-free. Age at surgery did not have an adverse effect on either seizure or cognitive outcomes.
Kossoff
Eric H
EH
Pediatric Epilepsy Center, Department of Neurology, The Johns Hopkins Medical Institutions, Baltimore, MD 21287, USA. ekossoff@jhmi.edu
Buck
Carol
C
Freeman
John M
JM
D000293:Adolescent; D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology / Q000523:psychology; D004351:Drug Resistance; D005260:Female; D007839:Functional Laterality / Q000502:physiology; D006801:Humans; D007223:Infant; D008297:Male; D009069:Movement Disorders / Q000209:etiology / Q000523:psychology; D019635:Neurosurgical Procedures / Q000009:adverse effects; D017060:Patient Satisfaction; D011183:Postoperative Complications / Q000453:epidemiology / Q000523:psychology; D012042:Registries; D012640:Seizures / Q000209:etiology / Q000601:surgery; D013341:Sturge-Weber Syndrome / Q000523:psychology / Q000601:surgery*; D011795:Surveys and Questionnaires; D016896:Treatment Outcome
D000927:Anticonvulsants