A 2-Year-Old Boy with Gelastic Seizures
A
2 year-old boy presented with spells consisting of abrupt laughter without provocation. His parents noted that these spells were accompanied by head deviation and twitching of the left side of the face. The spells lasted for less than 1 minute and he returned to normal activities immediately. The episodes were initially regarded as voluntary behavior to gain attention. There was a history of epilepsy in the mother and his maternal aunt. Neurologic examination and magnetic resonance imaging of the brain were normal. A video clip provided by the family helped to make the diagnosis (Video and Figure; Video available at www. jpeds.com). Subsequent electroencephalogram revealed that the episodes were indeed gelastic seizures originating from the right frontal and temporal regions of the brain. Gelastic seizures are characterized by bursts of laughter, which are often associated with other seizure types such as generalized convulsions or atonic seizures, and respond poorly to antiepileptic medications.1 Children with gelastic seizures commonly have hamartomas of the hypothalamus, precocious puberty, and developmental delay.2 How-
ever, our patient did not have an identifiable hamartoma. Gelastic seizures can also be manifestations of a seizure focus in the cingulate region of the frontal lobe or temporal lobe. The anterior cingulate region is involved in the motor act of laughter, and the temporal cortex is involved in processing of laughter’s emotional content. The hypothalamus plays a part in integrating these cortical centers with the brain stem, which is the final common pathway for the physiological responses involved in laughter, such as facial movements and vocalization. Therefore, lesions in any of these regions can give rise to gelastic seizures.3 Resection of the hypothalamic hamartoma, when present, can be curative for the seizures. Our patient is doing moderately well on a combination of anti-epileptic drugs. n Lalitha Sivaswamy, MD Department of Pediatrics
Young Ah Lee, MD Division of Pediatric Neurology
Preeti Borgohain, MD Division of Electroneurophysiology Children’s Hospital of Michigan Wayne State University School of Medicine Detroit, Michigan
References
Figure. Facial grimace suggestive of a smile during an episode of gelastic seizure.
1. Shahar E, Kramer U, Mahajnah M, Lerman-Sagie T, Goez R, Gross V, et al. Pediatric-onset gelastic seizures: clinical data and outcome. Pediatr Neurol 2007;37:29-34. 2. Sartori E, Biraben A, Taussig D, Bernard AM, Scarabin JM. Gelastic seizures: video-EEG and scintigraphic analysis of a case with a frontal focus; review of the literature and pathophysiological hypotheses. Epileptic Disord 1999;1:221-8. 3. Dericioglu N, Cataltepe O, Tezel GG, Saygi S. Gelastic seizures due to right temporal cortical dysplasia. Epileptic Disord 2005;7:137-41.
J Pediatr 2014;165:1067. 0022-3476/$ - see front matter. ª 2014 Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpeds.2014.07.030
1067
A
2 year-old boy presented with spells consisting of abrupt laughter without provocation. His parents noted that these spells were accompanied by head deviation and twitching of the left side of the face. The spells lasted for less than 1 minute and he returned to normal activities immediately. The episodes were initially regarded as voluntary behavior to gain attention. There was a history of epilepsy in the mother and his maternal aunt. Neurologic examination and magnetic resonance imaging of the brain were normal. A video clip provided by the family helped to make the diagnosis (Video and Figure; Video available at www. jpeds.com). Subsequent electroencephalogram revealed that the episodes were indeed gelastic seizures originating from the right frontal and temporal regions of the brain. Gelastic seizures are characterized by bursts of laughter, which are often associated with other seizure types such as generalized convulsions or atonic seizures, and respond poorly to antiepileptic medications.1 Children with gelastic seizures commonly have hamartomas of the hypothalamus, precocious puberty, and developmental delay.2 How-
ever, our patient did not have an identifiable hamartoma. Gelastic seizures can also be manifestations of a seizure focus in the cingulate region of the frontal lobe or temporal lobe. The anterior cingulate region is involved in the motor act of laughter, and the temporal cortex is involved in processing of laughter’s emotional content. The hypothalamus plays a part in integrating these cortical centers with the brain stem, which is the final common pathway for the physiological responses involved in laughter, such as facial movements and vocalization. Therefore, lesions in any of these regions can give rise to gelastic seizures.3 Resection of the hypothalamic hamartoma, when present, can be curative for the seizures. Our patient is doing moderately well on a combination of anti-epileptic drugs. n Lalitha Sivaswamy, MD Department of Pediatrics
Young Ah Lee, MD Division of Pediatric Neurology
Preeti Borgohain, MD Division of Electroneurophysiology Children’s Hospital of Michigan Wayne State University School of Medicine Detroit, Michigan
References
Figure. Facial grimace suggestive of a smile during an episode of gelastic seizure.
1. Shahar E, Kramer U, Mahajnah M, Lerman-Sagie T, Goez R, Gross V, et al. Pediatric-onset gelastic seizures: clinical data and outcome. Pediatr Neurol 2007;37:29-34. 2. Sartori E, Biraben A, Taussig D, Bernard AM, Scarabin JM. Gelastic seizures: video-EEG and scintigraphic analysis of a case with a frontal focus; review of the literature and pathophysiological hypotheses. Epileptic Disord 1999;1:221-8. 3. Dericioglu N, Cataltepe O, Tezel GG, Saygi S. Gelastic seizures due to right temporal cortical dysplasia. Epileptic Disord 2005;7:137-41.
J Pediatr 2014;165:1067. 0022-3476/$ - see front matter. ª 2014 Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpeds.2014.07.030
1067
![[Hypothalamic hamartoma revealing gelastic seizures].](/assets/img/hold.png)
