2008
DOI: 10.3171/foc/2008/25/9/e8
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Surgical management of hypothalamic hamartomas in patients with gelastic epilepsy

Abstract: Gelastic epilepsy (GE) associated with hypothalamic hamartomas (HHs) is now a well-characterized clinical syndrome consisting of gelastic seizures starting in infancy, medically refractory seizures with or without the development of multiple seizure types, and behavioral and cognitive decline. It has been postulated that the development of the HH-GE syndrome is a result of a progressive epileptic encephalopathy or secondary epileptogenesis, which is potentially reversible with treatment of the HH. A va… Show more

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Cited by 25 publications
(23 citation statements)
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“…2,5,6,11,13,18,25,26,28,30 Our case confirms that the epilepsy syndrome observed in adults with HH is different from the catastrophic epilepsy that develops in childhood. When epilepsy begins later, learning and behavioral problems are fewer, gelastic seizures are less prominent, and the epilepsy syndrome is usually restricted to 1 or 2 partial seizure types.…”
Section: Discussionsupporting
confidence: 73%
“…2,5,6,11,13,18,25,26,28,30 Our case confirms that the epilepsy syndrome observed in adults with HH is different from the catastrophic epilepsy that develops in childhood. When epilepsy begins later, learning and behavioral problems are fewer, gelastic seizures are less prominent, and the epilepsy syndrome is usually restricted to 1 or 2 partial seizure types.…”
Section: Discussionsupporting
confidence: 73%
“…2 For those in whom the first surgery fails, further medical treatment options remain limited; at times, a second surgery may improve seizure outcome. We retrospectively reviewed our surgical cases to document the success and complications of reoperation after a failed first surgery.…”
mentioning
confidence: 99%
“…GE is considered to be typically associated with hypothalamic hamartomas; additionally, it may also arise from temporal and frontal lobe tumors [71]. Therefore, surgical removal of the tumor is recognized as the main treatment for GE [70]. According to previous literature data, the rate of seizure freedom in patients with GE undergoing surgery can result in up to 50% [70], what's worse, the gelastic seizures are often refractory to anti-epileptic medications [72].…”
Section: Refractory Gelastic Epilepsymentioning
confidence: 98%
“…Gelastic epilepsy (GE) is a clinical syndrome characterized by gelastic seizures with onset in infancy, with or without the development of multiple seizure types, and cognitive and behavioral impairment [70]. GE is considered to be typically associated with hypothalamic hamartomas; additionally, it may also arise from temporal and frontal lobe tumors [71].…”
Section: Refractory Gelastic Epilepsymentioning
confidence: 99%