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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Gelastic epilepsy, or ictal laughter, is a relatively uncommon type of seizure which may occur singly or, more frequently, with other types of convulsions. Gelastic seizures have been observed to be associated with many different conditions, mainly hypothalamic hamartomas. We report on a patient whose ictal laughter was the only neurologic disturbance. Ictal video-EEG demonstrated seizure arising from the left frontal region with subsequent involvement of the contralateral homologous area and secondary generalization. MRI showed an enlarged left frontal horn of the lateral ventricle. Postictal SPECT, performed 6 min after the seizure had ended, showed hypoperfusion in the bilateral frontoparietal region and in both cerebellar hemispheres; the presence of this abnormality may be due to the spreading of the cortical epileptogenic focus and to the complex intercommunication between the frontal cortex and the cerebellar hemispheres. Interictal SPECT, in accordance with MRI features, demonstrated a left frontoparietal hypoperfusion. The neurofunctional features observed in the reported child could suggest that gelastic epilepsy originates in the frontal cortex. However, further studies are undoubtedly needed to define the pathogenetic mechanisms of ictal laughter.
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PMID:Gelastic epilepsy: video-EEG, MRI and SPECT characteristics. 933 71

Gelastic epilepsy, or laughing seizures, is a rare seizure manifestation often associated with hypothalamic hamartoma. This seizure type is well described in older children and adults, but has only rarely been reported in neonates, oftentimes recognized in retrospect when the children are older. We report a child diagnosed at 3 months of age with a large hypothalamic mass after evaluation for spells occurring since birth. The spells were characterized by bursts of hyperpnea, followed by repeated "cooing" respirations, giggling, and smiling. These spells were recognized soon after birth in the delivery room, and occurred at 15-20 minute intervals. They did not interrupt feeding and occurred during sleep. On referral to our center, the patient was noted to be thriving, with normal medical and neurologic examinations except for his spells. The laboratory evaluation was normal, as were endocrine and ophthalmologic evaluations. Neuroimaging was performed, with magnetic resonance imaging demonstrating a large 2.8-cm isodense, nonenhancing hypothalamic mass. Electroencephalogram was abnormal, demonstrating bi-frontal sharp and spike-wave discharges. Video-EEG did not demonstrate ictal discharges associated with the patient's spells. Single photon emission computed tomography (SPECT) demonstrated dramatic ictal uptake in the area of the tumor, with normalization during the interictal phase. Partial excision of hamartomatous tissue has minimally improved the spells. In conclusion, this patient manifested an unusual, early presentation of a rare seizure type. SPECT scanning confirmed the intrinsic epileptogenesis of the hamartoma, further justifying a surgical approach to such patients. Early surgical intervention is probably indicated in an attempt to minimize or prevent the cognitive and behavioral sequelae commonly seen with this seizure type.
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PMID:Utility of early single photon emission computed tomography (SPECT) in neonatal gelastic epilepsy associated with hypothalamic hamartoma. 1086 86

Gelastic epilepsy (GE) associated with hypothalamic hamartomas (HHs) is now a well-characterized clinical syndrome consisting of gelastic seizures starting in infancy, medically refractory seizures with or without the development of multiple seizure types, and behavioral and cognitive decline. It has been postulated that the development of the HH-GE syndrome is a result of a progressive epileptic encephalopathy or secondary epileptogenesis, which is potentially reversible with treatment of the HH. A variety of surgical options for the treatment of HHs exist, including open and endoscopic procedures, radiosurgery, interstitial radiotherapy, and stereotactic radiofrequency thermocoagulation. Surgical treatment can result in seizure freedom in up to 50% of patients and can be accompanied by significant improvements in behavior, cognition, and quality of life. Partial treatment of HHs may be sufficient to reduce seizure frequency and improve behavior and quality of life with less risk. A component of reversible cognitive dysfunction may be present in some patients with an HH-GE syndrome.
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PMID:Surgical management of hypothalamic hamartomas in patients with gelastic epilepsy. 1875 32

Gelastic seizure is a rare form of epilepsy defined as automatic bouts of laughter without mirth commonly associated with a hypothalamic hamartoma. Surgical treatment of hypothalamic hamartomas is associated with a high risk of complications because of the close vicinity of adjacent structures such as the optic tracts and mammillary bodies. This case was an 11-year-old girl who presented with gelastic seizure and complicated partial seizure. She developed gelastic seizure at a frequency of 10 bouts per day. She was found to have an elliptical mass close to the left hypothalamus. The signal intensity on magnetic resonance imaging (MRI) was consistent with hypothalamic hamartoma. The patient underwent surgical resection by a subtemporal approach. Pathological findings confirmed the diagnosis of hypothalamic hamartoma. Postoperative MRI demonstrated that the hypothalamic hamartoma was successfully resected. Twenty four months after surgery, complicated partial seizure in this patient has improved to Engel's class 1a and gelastic seizure has improved to Engel's class IIIa.
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PMID:[A successful surgical case of a hypothalamic hamartoma with gelastic seizure: a case report]. 1966 36

Gelastic epilepsy (GE) is an uncommon type of seizure disorder characterized by stereotyped, unprovoked, inappropriate ictal laughter. GE is most frequently associated with hypothalamic hamartoma, with onset almost invariably occurring during childhood. GE also occurs occasionally with temporal and frontal cortical seizure foci. We describe an unusual case of senescent-onset GE with a right frontal seizure focus. In addition to laughter, dysprosodia was a clinical feature. Clinical and electroencephalographic evidence of seizure activity ceased on levetiracetam, and the patient showed concurrent improvement in cognitive function. We review the evidence for the cerebral representation of laughter and prosody, and discuss issues bearing on the differential diagnosis and management of GE.
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PMID:Gelastic epilepsy and dysprosodia in a case of late-onset right frontal seizures. 1973 25

Gelastic seizure is an uncommon type of seizure which is characterized by recurrent bouts of unprovoked and stereotyped laughter. It is commonly observed in patients with hypothalamic hamartoma, while its association with other cerebral lesions is rare. The patient was a 15-year-old right-handed male. His chief complaints were recurrent onsets of laughter and unconsciousness for 10 years. On average, he had several to dozens of onsets per day and had failed most antiepileptic medications before admission. Presurgical evaluation included MRI, video-EEG, MEG and subdural electrode EEG. The results of MEG and subdural electrode EEG suggested that the epileptogenic focus was located on the lateral surface of the right frontal lobe, chiefly on the anterior part of the inferior frontal gyrus. The focus was removed under intraoperative ECoG monitoring which was consistent with the results of MEG and subdural electrode EEG. Histopathology revealed focal cortical dysplasia with balloon cells (type II). The patient kept seizure-free during the short-term follow up of 3 months. In the past literature, the medial frontal and basal temporal lobes, besides the hypothalamus, were thought to play major roles in the case of gelastic seizure. Our results suggest that the lateral surface of the frontal lobe might also be one part of the epileptogenic network in gelastic seizures. Removal of the epileptogenic focus under thorough pre-surgical evaluation might result in good seizure control in patients with gelastic seizures.
Seizure 2011 May
PMID:Surgical outcome of gelastic epilepsy of frontal lobe origin: a case report. 2120 13

Gelastic epilepsy are focal seizures manifesting as recurrent brief seizures starting as laughter or grimaces. They are most commonly associated with other types of seizures and can be secondary to infectious, malformative, metabolic, or neoplastic processes involving the central nervous system. We report on an 18-month-old girl who presented since the age of 2 months with multiple, recurrent, unprovoked episodes of stereotypical laughter. Brain magnetic resonance study revealed an hypothalamic hamartoma. Endoscopic tumor disconnection of the hamartoma resulted in rapid resolution of neurological symptomatology.
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PMID:Gelastic seizures due to hypothalamic hamartoma: rapid resolution after endoscopic tumor disconnection. 2282 43

Gelastic epilepsy has been reported to originate from various conditions, particularly from hypothalamic hamartoma (HH). In the present study, we report a patient with gelastic seizures (GSs), followed by complex partial and tonic-clonic seizures. Magnetic resonance imaging (MRI) revealed a rare combination of HH and partial agenesis of the corpus callosum (ACC). Following resectioning of the HH, the seizures were reduced, but not fully controlled, with medication by the one year follow-up. HH and partial ACC patients may experience seizures; the seizures in the case presented in this study may have originated from HH, partial ACC or both. Considering the fact that seizure frequency reduced following surgery, they may have mainly occurred from HH. Additionally it was considered to be likely that the seizures following surgery were due to secondary epileptogenesis, partial ACC, or both.
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PMID:Gelastic epilepsy in combination with hypothalamic hamartoma and partial agenesis of the corpus callosum: A case report and review of the literature. 2425 88

Gelastic seizures, characterized by epileptic laughter, are rare and the majority is associated with hypothalamic hamartomas. We report a case with cryptogenic Gelastic seizure (without hypothalamic hamartoma), as the MRI was normal and, EEG and clinical data suggested a focal origin of the seizures.
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PMID:Cryptogenic gelastic epilepsy: a pediatric case vignette. 2478 18

Gelastic seizure was first described by Trousseau in 1877 and comes from the Greek word gelos (laughs), as laughter is the main feature [1]. Normal laughter is a reactive emotional behaviour and motor action that involves the limbic system, hypothalamus, temporal cortex, and several regions of the brainstem. A female patient, six years old, left-handed, with gelastic seizures, uncontrolled despite being treated with two antiepileptic drugs at high doses, was treated. A simple axial tomography was done, where a hypodense lesion that shapes the inner table of the skull temporal level was observed; later, magnetic resonance imaging was requested, better characterising an intraxial lesion in the right second temporal gyrus cystic appearance.
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PMID:Child with temporal lobe hamartoma: A to Z images and a case report. 2496 88


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