A review on the management of epilepsy associated with hypothalamic hamartomas.
BACKGROUND
Hypothalamic hamartomas are rare congenital malformations located in the region of the tuber cinereum and third ventricle. Patients may be asymptomatic, but the usual presentation is gelastic seizures, precocious puberty, and/or developmental delay.


METHODS
Without surgical intervention, the gelastic seizures, which are typically present in childhood, may progress to other seizure types, including generalized epilepsy, and are generally refractory to antiepileptic drugs.


CONCLUSIONS
This review will discuss the clinical and electrophysiologic aspects of these lesions, as well as treatment options, including surgery, endoscopy, and radiosurgery.
Frazier
James L
JL
Department of Neurosurgery, Meyer 8-161, Johns Hopkins Hospital, 600 North Wolfe Street, Baltimore, MD 21287, USA. jfrazier@jhmi.edu
Goodwin
C Rory
CR
Ahn
Edward S
ES
Jallo
George I
GI
D001519:Behavior; D001918:Brachytherapy; D017115:Catheter Ablation; D003072:Cognition Disorders / Q000150:complications; D004724:Endoscopy; D004827:Epilepsy / Q000150:complications* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy*; D006222:Hamartoma / Q000150:complications* / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D007031:Hypothalamus / Q000473:pathology / Q000503:physiopathology*; D009493:Neurosurgery / Q000379:methods; D016634:Radiosurgery; D012640:Seizures / Q000150:complications / Q000473:pathology / Q000503:physiopathology; D055536:Vagus Nerve Stimulation