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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An update in the clinical presentation, diagnosis and treatment”. The endocrinology of hypothalamic hamartoma surgery for intractable epilepsy. Laughter-like vocalization is epilepy combined with facial contraction in the form of a smile. Since our cohort did not include patients with precocious puberty without seizures, or cognitive impairment without seizures, and because the issues of precocious puberty and cognitive impairment were not the epile;sy focus of our study, we did not perform systematic comparison of the structural abnormalities in patients with and without precocious puberty or cognitive impairment.

Multimodal imaging in familial FTLD: This difference has been explained by the nature of the HH, which is a lesion that has a tendency to displace rather than replace normal structures Freeman et al b. Epigastric discomfort, fear, oral and bimanual automatisms, axial movements and complex bi-pedal movements are frequently reported during complex partial seizures in patients with HH Kuzniecky et al ; Maixner The anatomical dimensions of the hypothalamic hamartomas were also measured in three planes: Hence, orexin neurons are apparently associated with laughter, and have descending projections that normally prevent the onset of atonia.

Intrinsic epileptogenesis of hypothalamic hamartomas in gelastic epilepsy.

A gelastic seizure is defined as sudden occurrence epileepsy emotions in the form of a laugh or a cry. Gelastic epilepsy in tumours of the hypothalamic region.

If the child has epilepdy seizures epjlepsy precocious puberty which means that they go into puberty very early, usually under 10 years of agethen it is likely that the child will be found to have a hypothalamic hamartoma a hamartoma in the hypothalamus part of the brain.


Hypothalamic hamartomas and ictal laughter: Vagal nerve stimulation has been used, but the results are equivocal Murphy et al This is likely due to the fact that surgical treatment of hypothalamic hamartomas has been available at specialized centres only over the past 10 years, and consequently treatment could be offered recently to relatively large numbers of previously untreated patients with hypothalamic hamartomas.

Gelastic Epilepsy | Epilepsy Queensland

Evolution and post operative resolution. The form of epilepsy is; however, very rare and out of every 1, children who experience a form of epilepsy, only 1 or 2 will experience gelastic epilepsy.

All patients except one improved clinically. Sign In or Create an Account. Partial epileepsy evolving to infantile spasms. Usually the seizure gelastif around 5 to 60 seconds. Some studies have suggested that small HHs can have less severe seizures, less cognitive problems and a lower frequency of precocious puberty Sturm et al ; Striano et al Gelastic epilepsy refers to a type of epilepsy in which the seizures are ‘gelastic’, gelastikos being the Greek word for laughter.

Citing articles via Web of Science Transcallosal resection of hypothalamic hamartoma for intractable epilepsy. In patients who were referred after the age of 12 years, we used clinical history rather than formal endocrinological evaluations to determine if they had suffered from precocious puberty. Subsidence of seizure induced by stereotactic radiation in a patient with hypothalamic hamartoma. Utility of early single photon emission computed tomography SPECT in neonatal gelastic epilepsy associated with hypothalamic hamartoma.

They might; however, improve after surgical or radiotherapy gelastif if the cause is due to a hamartoma. Despite the use of higher dose and several combinations of antiepileptic drugs AEDsseizure freedom or good seizure control is rarely achieved in this group of eppilepsy Harvey and Freeman These patients exhibit stereotypical behaviour of mirthless laughter during their ictal events.

All patients had been thoroughly evaluated for their IQ or developmental quotient DQas described previously Prigatano et al. MR imaging and spectroscopic study of epileptogenic hypothalamic hamartomas: Mathieu et al The mean lesion volume was measured as 1.


Cognitive gelasgic in children with gelastic seizures and epilepsh hamartoma.

Parallel to previous observations that anterior contact with pituitary stalk may contribute to the development of precocious puberty, in our cohort, all cases with precocious puberty had lesions that were significantly larger in the anterior extent of the lesions.

In most cases gelastic seizures are associated with other seizure types. At times, older children might complain of a warning or aura beforehand, although they are not always able to describe exactly what the warning is like.

Cognitive functions in juvenile and adult patients with gelastic epilepsy due to hypothalamic hamartoma. In patients with GTCS the EEG frequently shows an increase in the amount of interictal generalized spike wave activity during wakefulness and sleep and it is possible to see secondary bilateral synchrony.

Unfortunately, the behavioral and learning issues rarely respond to medical treatment. The episodes of laughter may be confused with emotional or behavioral disorders and this might delay achievement of a diagnosis, something that is especially likely to occur in younger children.

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Once again, there are very few areas in the world that offer this treatment. Bicarbonate contributes to GABAA receptor-mediated neuronal excitation in surgically resected human hypothalamic hamartomas. The episodes can also be confused with behavioral and emotional disorders.

When the syndrome appears in adults, the prognosis may be better, with less severe epilepsy and better neurophysiological outcomes. GS usually occur with high frequency and periodicity, especially in children.

This may cause learning disabilitiesand faulted cognitive function as well. Despite the popularity of this therapy the main disadvantage of the procedure is the delay of its effect. Status gelasticus associated with levetiracetam as add-on treatment.