J Korean Epilepsy Soc.  2013 Jun;17(1):8-16.

Clinical Manifestations and Treatment of Hypothalamic Hamartoma

• Lee J
• Yu HJ
• Kim JY
• Kim JH
• Lee JI
• Shin HJ
• Lee M
• Lee J

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.

Keyword

Hypothalamic hamartoma; Gelastic seizure; Precocious puberty; Gamma-knife radiosurgery

MeSH Terms

Brain
Epilepsy
Hamartoma
Humans
Magnetic Resonance Imaging
Medical Records
Puberty, Precocious
Radiosurgery
Retrospective Studies
Seizures