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Study into major surgery in children with Sturge-Weber syndrome

11 Dec 2002

Researchers report that hemispherectomy – a procedure in which half the brain is removed - may reduce or eliminate severe seizures even in older children with a rare disorder associated with epilepsy.

The findings are published in the December issue of the journal Neurology.

Contrary to results of previous studies, the study at Johns Hopkins Children's Center in Baltimore found that in children with Sturge-Weber syndrome, delaying hemispherectomy even for years had no apparent effect on seizure control or learning ability. Some 80 per cent of Sturge-Weber patients develop epilepsy.

Dr Eric Kossoff, a paediatric neurologist at the Children's Center, said:

"In fact, older patients were statistically more likely to be seizure-free after surgery. However, in general, the child's age at surgery did not have an adverse effect on either their intellectual abilities or seizure reduction."

Sturge-Weber Syndrome is a rare condition in which the presence of a birth mark (port wine stain), usually on one side of the face, is associated with an abnormality of the brain caused by abnormal blood vessels on the surface of the brain. This abnormality usually results in seizures or other problems.

More than 80 per cent of patients were reported to be seizure-free following the surgery and more than half were off anticonvulsants. The type of hemispherectomy that was performed, or the amount of brain matter actually removed, did not influence the child's seizure outcome.

"Most interestingly, we found the child's cognitive skills were not impacted by the child's age at operation or delay of surgery. This is contrary to all other previous studies on Sturge-Weber syndrome and hemispherectomy, which emphasized early surgery to avoid cognitive decline," said Kossoff. "However, there was a trend toward poorer cognitive outcomes if seizures persisted after surgery."