Gelastic Seizures: Two Case Presentations
A Recep, S Ilhan, à Türk
Keywords
eeg, elastic seizures, g, hypothalamic hamartomas, laughter epilepsy, temporal lobe
Citation
A Recep, S Ilhan, à Türk. Gelastic Seizures: Two Case Presentations. The Internet Journal of Neurology. 2006 Volume 6 Number 1.
Abstract
Gelastic seizures are rarely observed, in their etiology we mostly find hypothalamic hamartomas, very rarely we cannot identify any cause in the etiology. Uncontrolled episodes of laughter were the reasons for the admittance of our cases to our outpatient department. In one case there were findings of atrophy on right temporal region while we could not identify any cause in the other. Here we discuss both cases under the light of the data in the literature.
Introduction
Gelastic seizures that are characterized by uncontrolled episodes of laughter have a very limited share within the spectrum of all epileptic fits. They can sometimes present themselves together with other epileptic syndromes; patients with early onset gelastic seizures are generally identified to have a hamartoma (1, 2, 3, 4). They can be of temporal lobe origin or extratemporal or cryptogenic. We found two cases with gelastic seizures worth presenting as it is a rare condition.
Case presentation
Case 1
A 21-year old male patient admitted to our outpatient department with the complaints of involuntary uncontrollable episodes of laughter of irregular pattern that have been present for the last 4-5 years. He complained of not being taken seriously by his friends and his family because of these episodes and talked of being confronted with humiliating situations. He said that he had the continuous effort of making jokes to cover up this condition. In his history there were 2-3 febrile convulsions at the age of 2-3. He had normal physical and neurological examination results. In the EEG that was recorded, there was paroxysmal sharp wave activity. In cranial MR imaging, there was thinning of the right fornix, atrophy of the mamillary body and diminishment in the volume of the right amygdale. The patient was administered carbamazepine. There was partial improvement of the seizures.
Case 2
A 14 year old male patient was referred to our outpatient department because of uncontrolled involuntary episodes of laughter. We learned that his complaints have been present for the last 2-3 years but aggravated recently. He mentioned being warned by his friends and teachers continuously because of this condition and that this irritated him. The patient had changes of mood starting from age 4 onwards he was making jokes at home and school and trying to entertain the others with funny behaviors. He at the same time had nocturnal enuresis. In his history he had febrile convulsions at the age of 1. The parents were first degree relatives. His physical and neurological examination were both normal. The EEG revealed paroxysmal and frequent sharp wave activity. (Figures 1, 2). Cranial MR imaging was normal. His IQ was calculated as 78.
Figure 1
The patient was treated with 500 mg valproate. His complaints were found to have decreased in his control visit a month later. In the EEG obtained three months later, despite the fact that there were generalized sharp wave paroxysms, when compared with the previous EEG the epileptic activity was found to have decreased. (Figures 3, 4).
Figure 2
Discussion
Neuronal control of the laughter is quite complex. Frontal and temporal lobes, limbic system and brain stem all play a role in the generation of laughter. In certain instances we talk about pathological laughter. Amongst these we can list pseudobulbar palsy, emotional lability, behavioral disturbances, and gelastic seizures by themselves or together with other forms of epilepsy (5, 6).
Gelastic seizures (uncontrolled episodes of laughter) are rare and are mostly seen together with other forms of epilepsy. Hypothalamic hamartomas have been mentioned in its etiology. Furthermore, pituitary tumors, astrocytomas of the mamillary body, tumors of the temporal lobe or pineal region, papillomas of the third ventricle, encephalitis, meningitis, lipid storage diseases and neuronal migration anomalies have been reported. Rarely, no cerebral anomaly can be identified in the etiology (7, 8, 9).
Gelastic epilepsy cases can present with uncontrolled episodes of laughter, other behavioral disturbances, mental hindrance and failure at school (1). Our first case had had behavioral abnormalities while the second had failure at school, behavioral disturbances and mental problems. In cranial imaging, the first case had mild atrophy of the right temporal region while no lesion was identified in the second one.
Gelastic seizures are mostly seen together with temporal lobe epilepsies and are rarely together with seizures that originate from extratemporal foci. For gelastic epilepsy, together with paroxysmal discharges in interictal EEG, different EEG patterns have been reported: like focal abnormalities in frontal, temporal and parasagittal regions. In the presence of a lesion, different EEG findings have been reported depending on the localization of the lesion (9, 10, 11). We have observed paroxysmal activities in the EEGs of our patients.
In SPECT studies aimed at identifying the etiopathogenesis, hyperperfusion has been identified at different localizations (12, 13). There was hypoperfusion in hamartomas during interictal period, while there was hyperperfusion during the ictal period. In certain cases there was normal perfusion. In other studies, hyperperfusion was identified in temporal and frontal cortexes. Although there was a hamartoma in one case, only right cingulated gyrus was shown to be affected (14). In conclusion, other pathways might also be influential in the generation of gelastic seizures.
Correspondence to
Recep ALP Address: Firat Mah. Erzurum Cad. Firat Apt. Kat:3 D.14 –AGRI Phone:+904722157272 e-mail: recep.alp@gmail.com