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GELASTIC EPILEPSY PDF

A gelastic seizure, also known as “gelastic epilepsy”, is a rare type of seizure that involves a sudden burst of energy, usually in the form of laughing or crying. Information regarding gelastic epilepsy, a rare form of epilepsy that involves laughter or crying and is more common in boys than girls. What is Gelastic Epilepsy? Gelastic epilepsy refers to a type of epilepsy in which the seizures are ‘gelastic’, gelastikos being the Greek word for laughter.

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However, we found a clear difference in the length of time from the diagnosis to surgery between females and males even though the age of onset of seizures was not significantly different between the two groups. The hypothalamic hamartoma lesions are focal congenital tumours composed of cytologically normal, small and large neurons, which are organized in poorly demarcated clusters of variable size and density Coons et al. Some selected series have reported typical temporal lobe seizures with electrographic changes in the temporal area and where the consideration of a temporal lobectomy was performed Cascino et al Homma and colleagues recently published a series of five patients with small HHs and intractable GS.

If a child also has precocious puberty, this may gelastlc treated with hormones, or hormone-like medications. Clin Pediatr Phila ; Other small series also suggested the efficacy of gamma knife in the treatment of patients with GS associated with HH Barajas et al ; Selch et al ; Mathieu et al Electrophysiological properties and subunit composition of GABAA receptors in patients with gelastic seizures and hypothalamic hamartoma.

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Some associations have been shown between the localization of the HH and the clinical profile, although these are not constant. It has been suggested that gelastic epilepsy can be viewed as a spectrum of conditions with various degrees of severity ranging from previously normal children with gelastic epilepsy evolving towards a catastrophic symptomatic generalized epilepsy or partial epilepsy with cognitive impairment Striano et al.

Evolution and post operative resolution. If the child also has precocious puberty, then this can be treated with hormones or hormone-like medicines. The anti-epileptic drugs AEDs used to treat focal seizures may also be effective in treating gelastic epilepsy.

Regis and colleagues enrolled 60 patients with HH treated with gamma knife between and Also in children it is possible to see ictal and interictal generalized polyspike waveform discharges Freeman et al a. The rationale for using mammillary bodies as the point of reference is the ease with which these nuclei can be identified.

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Reported focal motor features including head and eye version, asymmetric tonic posturing, tonic or clonic lower facial contraction, and limb jerking. People with Gelastic Epilepsy may also have other types of seizures either immediately after these gelastic seizures or at other times. Skip to main content.

Human epileptogenesis and hypothalamic hamartomas: Translation and analysis of a cuneiform text forming part of a babylonian treatise on epilepsy. Ninety-one percent of HHs in patients with isolated precocious puberty revealed a parahypothalamic position without affecting the third ventricle.

The laughter commonly lasts less than a minute and is followed by signs that are more usually recognized with focal seizures.

Electrophysiological properties of human hypothalamic hamartomas.

Subsidence of seizure induced by stereotactic radiation in a patient with hypothalamic hamartoma. In our cohort, 32 patients had gelastic epilepsy-only seizures and 68 patients had gelastic epilepsy-plus, i.

Gelastic seizure – Wikipedia

Treatment options for gelastic epilepsy due to hypothalamic hamartoma: Same observations have been reproduced in other series of patients with GS and HH Brandberg et al ; Maixner Role of the hypothalamic hamartoma in the genesis of gelastic fits a video-stereo-EEG study.

Finally some authors have presented convincing reports of GS from the temporal area. While the mammillary bodies themselves project heavily to the anterior nucleus of the thalamus [which in turn has access to greater limbic lobe of the brain and its target structures Heimer and Van Hoesen, ], there are other posterior hypothalamic nuclei e. However, although a computed tomography scan may be useful in diagnosing the cause of a seizure, in the case of a hypothalamic hamartoma, magnetic resonance imaging is the tool of choice due to the cerebrospinal fluid which defines these masses.

Patients with intractable epilepsy associated with HHs should be investigated with intracranial recording in order to clarify the seizure onset in the HH. Most patients had video-EEG monitoring at the referring centres. The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma. Bicarbonate contributes to GABAA receptor-mediated neuronal excitation in surgically resected human hypothalamic hamartomas.

Sterotactic radiofrequency thermocoagulation is a safe procedure and despite of some postoperative transitory hypothalamic symptoms, no permanent neurologic deficits have been reported Kuzniecky et al ; Parrent ; Homma et al These findings beg the question of why hamartomas cause seizures with the specific ictal behaviour of laughter?

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Intrinsic epileptogenesis of hypothalamic hamartomas in gelastic epilepsy.

Gelastic seizure

New lessons from an experiment of nature. The patient was treated successfully with carbamazepine. Treatment of patients with gelastic seizures and hypothalamic hamartomas GS are characteristically refractory to medical treatment and the progressing epileptic process eventually leads to deterioration of cognitive and behavioral function Palmini et al Status gelastci after temporal lobectomy: This may cause learning disabilitiesand faulted cognitive function as well.

Transcallosal resection of hypothalamic hamartoma for intractable epilepsy. Gelastic and other types of seizures are often times very hard to control. HHs are rare tumors associated with the triad of gelastic epilepsy, precocious puberty, and developmental delay.

Progressive cognitive deficits and the presence of important psychiatric comorbidity are widely described. Gepastic this occurs between the ages of 4 to 10 years and involves the presence of multiple types of seizures, including complex partial seizures with or without secondary generalization, generalized tonic-clonic seizures and drop attacks Brandberg et al ; Striano et al The episodes can also be confused with behavioral and emotional disorders.

Gelastic Epilepsy | Epilepsy Queensland

We are hopeful that these findings will motivate future research towards understanding the precise nuclear anatomy of hypothalamic hamartomas and the routes by which they propagate ictal discharges to cortical and subcortical networks. GS are characteristically refractory to medical treatment and the progressing epileptic process eventually leads to deterioration of cognitive and behavioral function Palmini et al Electrophysiology and type of seizures in patients with gelastic seizures associated with HH Scalp EEG is very limited in order to show epileptiform activity due to the deep localization of the lesion and the complex connections of the HHs Maixner The endocrinology of hypothalamic hamartoma surgery for intractable epilepsy.

Retrospective analysis of surgical treatment outcomes for gelastic seizures: If a child has gelastic seizures and, ‘precocious puberty,’ which means they go into puberty very early – usually before the age of 10, then it is likely that they will epilwpsy found to have a, ‘hypothalamic hamartoma,’ or a hamartoma in the hypothalamus part of their brain.

These HHs do not disturb the hypothalamic region and predominantly produce precocious puberty.