TY - JOUR
T1 - Lennox-Gastaut syndrome symptomatic to hypothalamic hamartoma
T2 - Evolution and long-term outcome following surgery
AU - Pati, Sandipan
AU - Deep, Aman
AU - Troester, Matthew M.
AU - Kossoff, Eric H.
AU - Ng, Yu Tze
N1 - Funding Information:
Disclosures: E. H. Kossoff is a paid consultant for Atkins Nutritionals, Inc., Nutricia, Inc., Eisai, Inc. and his research is supported by Nutricia, Inc. and NIH. Y. T. Ng is a paid consultant for Lundbeck, Inc., and is on the speakers' bureau for UCB Pharma, Lundbeck Inc., Supernus Pharmaceuticals Inc. and Cyberonics Inc.
PY - 2013/7
Y1 - 2013/7
N2 - Background: Lennox-Gastaut syndrome is a catastrophic childhood cryptogenic or symptomatic epilepsy. Hypothalamic hamartomas cause refractory epilepsy often consistent with Lennox-Gastaut syndrome. Methods: Children with Lennox-Gastaut syndrome were defined by a triad of multiple generalized seizure types, slow spike-and-wave on EEG, and mental retardation. Results: Twenty-one of 159 hypothalamic hamartoma patients (14%) met the diagnostic criteria of Lennox-Gastaut syndrome. The median age of patients at epilepsy onset was 0.9 years (range, birth to 9 years). Six of the 21 patients (28%) had preceding infantile spasms. All patients underwent different surgical approaches, including endoscopic, transcallosal, orbitozygomatic resections, and radiosurgery treatment. Five of the 21 (24%) were seizure free with an additional 9 (42%) having at least >90% seizure reduction. Only 1 patient was not effectively treated (<50% seizure reduction). Eighty-eight percent of parents reported improvement in behavioral functioning. Shorter duration of epilepsy prior to surgery was a significant predictor of surgical outcome. Conclusions: Patients with Lennox-Gastaut syndrome symptomatic to hypothalamic hamartomas have better postsurgical outcome due to other etiologies compared with cryptogenic and symptomatic Lennox-Gastaut syndrome patients. However, compared with overall hypothalamic hamartomas postsurgical outcomes, this cohort was less favorable. Earlier surgery may lead to better outcomes.
AB - Background: Lennox-Gastaut syndrome is a catastrophic childhood cryptogenic or symptomatic epilepsy. Hypothalamic hamartomas cause refractory epilepsy often consistent with Lennox-Gastaut syndrome. Methods: Children with Lennox-Gastaut syndrome were defined by a triad of multiple generalized seizure types, slow spike-and-wave on EEG, and mental retardation. Results: Twenty-one of 159 hypothalamic hamartoma patients (14%) met the diagnostic criteria of Lennox-Gastaut syndrome. The median age of patients at epilepsy onset was 0.9 years (range, birth to 9 years). Six of the 21 patients (28%) had preceding infantile spasms. All patients underwent different surgical approaches, including endoscopic, transcallosal, orbitozygomatic resections, and radiosurgery treatment. Five of the 21 (24%) were seizure free with an additional 9 (42%) having at least >90% seizure reduction. Only 1 patient was not effectively treated (<50% seizure reduction). Eighty-eight percent of parents reported improvement in behavioral functioning. Shorter duration of epilepsy prior to surgery was a significant predictor of surgical outcome. Conclusions: Patients with Lennox-Gastaut syndrome symptomatic to hypothalamic hamartomas have better postsurgical outcome due to other etiologies compared with cryptogenic and symptomatic Lennox-Gastaut syndrome patients. However, compared with overall hypothalamic hamartomas postsurgical outcomes, this cohort was less favorable. Earlier surgery may lead to better outcomes.
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U2 - 10.1016/j.pediatrneurol.2013.03.016
DO - 10.1016/j.pediatrneurol.2013.03.016
M3 - Article
C2 - 23827423
AN - SCOPUS:84880073988
SN - 0887-8994
VL - 49
SP - 25
EP - 30
JO - Pediatric Neurology
JF - Pediatric Neurology
IS - 1
ER -