Very rare disease. It presents as an epileptic seizure, the only or main sign of which is inappropriate, uncontrolled laughter ("laughing seizure"). They are highly associated with abnormal cognitive development and behavioral problems. It is characterized by electrical discharges in the area of the brain responsible for laughter (medial temporal and cingulate regions). It is usually associated with a tumor (hamartoma) of the hypothalamus (see Hypothalamic hamartomas and Pallister Hall syndrome) or, more rarely, of frontal origin.

This form of epilepsy is generally drug-resistant, but responds well to videoendoscopic neurosurgery: thermocoagulation or disconnection of the hamartoma or hypothalamus, depending on the size of the hamartoma.

Anesthetic implications:

drug-resistant epilepsy; neurosurgery in the third ventricle

References : 

-        Oehl B, Brandt A, Fauser S, Bast T, Trippel M, Schulze-Bonhage A.
Semiologic aspects of epileptic seizures in 31 patients with hypothalamic hamartoma.
Epilepsia 2010 ; 51:2116-23

-        Wang S, Zhao M, Li T, et al.
Stereotactic radiofrequency thermocoagulation and resective surgery for patients with hypothalamic hamartoma.
J Neurosurg. 2020;1:1-8

-        Van Dong H,Van Dinh T, Dinh-Hoa N, Van Vu H5, Tuan Anh N et al.
Successful endoscopic disconnection for hypothalamic hamartomas in a child with gelastic seizures: an unusual case in a resource-scare setting.
International Medical Case Reports Journal 2020:13 425-9

Updated: January 2024