J Korean Pediatr Soc.  1996 Mar;39(3):436-441.

A Case of Hypothalamic Hamartoma with Gelastic Seizure Only

• Oh SW
• Cho JH
• Kim SW

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.

Keyword

Arteriovenous malformation; Spine; Epidural hematoma Lumbar puncture; Paraplegia

MeSH Terms

Anticonvulsants
Arteriovenous Malformations
Cleft Palate
Electroencephalography
Hamartoma*
Humans
Intellectual Disability
Magnetic Resonance Imaging
Optic Chiasm
Paraplegia
Puberty, Precocious
Seizures*
Spine
Tetralogy of Fallot
Anticonvulsants