ISSN 2234-5531
 
 
Original Articles
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Journal of K orean Epilepsy Society 2013 June;17(1):8-16.
Published online 2013 October 14
Copyright ⓒ 2010 Journal of Korean Epilepsy Society
시상하부 과오종의 임상 양상과 치료
이지원1⋅유희준1⋅김재연1⋅김지혜2⋅이정일3⋅신형진3⋅이문향1⋅이지훈1
성균관대학교 의과대학 삼성서울병원 1소아청소년과, 2영상의학과, 3신경외과
Corresponding Author: Jeehun Lee, M.D., PhD. ,Tel: +82‐2‐3410‐0, Fax: +82-2‐3410‐0043, Email: jhlee0101@skku.edu
ABSTRACT
Purpose: The aim of this study was to describe the clinical manifestations and radiologic characteristics of patients with hypothalamic hamartoma (HH) and to delineate various treatment modalities.
Methods: We retrospectively reviewed the medical records of 11 patients with HH at Samsung Seoul Hospital between 1997 and 2013.
Results: Mean age of diagnosis was 2.7 years. Seven patients had intractable epilepsy, seven exhibited precocious puberty, and four had both. Gelastic seizure was the most common seizure and was intractable to medications. The patients with the intrahypothalamic type (18.2%) had no precocious puberty, while those with the parahypothalamic type (36.4%) didn’t have seizures. Patients (n=6) with intractable epilepsy underwent gamma-knife radiosurgery (GKS); five of these patients responded to this treatment.
Conclusions: The most frequent presenting symptoms of HH were gelastic seizure and precocious puberty. The location and size of tumor defined by brain magnetic resonance imaging affected presenting symptoms and the selection of surgical treatment. GKS can be considered if the patients showed intractable epilepsy and the size of tumor is less than 2 cm in diameter.
Keywords: Hypothalamic hamartoma, Gelastic seizure, Precocious puberty, Gamma-knife radiosurgery

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