All issues > Volume 39(3); 1996

Case Report

J Korean Pediatr Soc. 1996;39(3):431-435. Published online March 15, 1996.

A Case of Hypothalamic Hamartoma with Gelastic Seizure Only

Se Wook SW Oh¹, Je Ho JH Cho¹, Sang Woo SW Kim¹

¹Department of Pediatrics, Seoul Paik Hospital, College of Medicine, Inje University, Seoul, Korea

Abstract

Hypothalamic hamartoma is a congenital anomaly presenting precocious puberty, gelastic seizure and mental retardation. Other possible accompanying anomalies are cleft palate, tetralogy of Fallot, skeletal malformation and anal malformation. But the patient describing here manifested gelastic seizure only with isosignal mass of 1.5cm in diameter situated just behind optic chiasm in T1WI and T2WI of MRI. His EEG showed multifocal spike and sharp waves at left frontal and central area with asymmetric sleep spindles. The seizure has been refractory to conventional antiepileptic drugs. He is now on Lamotrigine, a newly developed antiepileptic drug available.

Keywords :Hypothalamic hamartoma, Gelastic seizure