Gelastic epilepsy: symptoms, causes and treatment

This epilepsy produces a characteristic symptom: a "hollow" and involuntary laughter.

We all know the beneficial effects of laughter. Laughing helps us to relax and feel better, and can even relieve pain. However, this is not always the case.

In the movie "Joker", starring Joaquin Phoenix, we can see how his character, Arthur Fleck, shows a sudden and loud laughter that he can't seem to control and that makes him have real problems living and communicating with others.

The disease underlying the uncontrolled laughter of the protagonist of the film is none other than gelastic epilepsy, a very rare type of epilepsy characterized by the manifestation of epileptic seizures in the form of laughter.

Throughout the article we will talk about gelastic epilepsyThe following is a summary of the causes of epilepsy, the symptoms it causes, and how to diagnose and treat this disorder.

What is gelastic epilepsy?

Gelastic epilepsy is a type of epilepsy in which "gelastic" seizures occur.. This term comes from the Greek "gelastikos" which means laughter in our language, so it is precisely this inappropriate laughter that manifests itself in gelastic seizures. This disease is slightly more common in boys than in girls, although it has a low incidence and occurs in 1 or 2 out of every 1,000 children with this brain disorder.

The most common areas of the brain that give rise to gelastic seizures are the hypothalamus (a region with an important role in various visceral autonomic and endocrine functions), the temporal lobes and the frontal lobes.

Causes

A common cause of this type of epilepsy is usually the appearance of a tumor in the hypothalamus, which can be of two types: a hamartoma or an astrocytoma.This can be of two types: a hamartoma or an astrocytoma.

A hamartoma is a benign (non-cancerous) growth composed of an abnormal mixture of cells and tissues normally found in the area of the body where the growth occurs. And an astrocytoma is a nervous system tumor that grows from astrocytes, a type of glial cell (nervous system support cells).

Most of these tumors are benign. This means they may grow very slowly and not spread to other parts of the brain or body. However, if a child has gelastic seizures and precocious puberty, it is more likely that a hypothalamic hamartoma can be detected.

In addition, it is common for older children who have gelastic epilepsy caused by a hypothalamic hamartoma to also have learning and behavioral problems that often worsen in middle or late adolescence.

Symptoms

The seizures of gelastic epilepsy can begin at any age, but usually begin before age 3 or 4. The seizures usually begin with laughter that is often described as "hollow" or "hollow" and not very pleasant, although sometimes and not very pleasant, although it may sometimes sound like a child's normal laughter.

The laughter comes on suddenly, appears for no obvious reason, and is usually completely out of place. Older children can sometimes predict the onset of seizures by experiencing feelings of fear or unpleasant smells and tastes just before they occur. These sensations have also been described in other types of epilepsy and are known as "aura", a peculiar sensation that warns of the proximity of epileptic seizures.

The laughter usually lasts 30 to 45 seconds and stops suddenly.. It may then be followed by signs that are seen more frequently in focal seizures, such as side-to-side movements of the eyes and head, automatisms (such as lip smacking, mumbling or hand movements), alterations of consciousness or lack of responsiveness to those around them. These signs may last from seconds to many minutes and then stop.

Gelastic seizures may occur along with other types of seizures, either immediately after or at other times.either immediately after or at other times. These include tonic-clonic seizures, which involve whole-body rigidity and jerking of the arms and legs with loss of consciousness; and atonic seizures, in which there is weakness of all muscles of the body.

Diagnosis

When making a diagnosis in gelastic epilepsy, a detailed history of the patient should be sought. a detailed history and a description of the seizures the child has had should be sought. Episodes of laughter may be confused with behavioral or emotional disorders, even in children with attention difficulties or features of autism spectrum disorder. Therefore, the latter may delay diagnosis and is more likely to occur in young children.

Occasionally, a videotape with sound of the child's episodes can be very helpful in ruling out or confirming the disorder. However, it is necessary to perform an EEG, it is necessary to perform an electroencephalogram. to detect focal and generalized abnormalities (sharp waves, spikes or spikes and slow waves).

A brain scan is also necessary for a correct diagnosis to determine whether or not there are tumors located in the hypothalamus or in other brain areas, such as the temporal or frontal lobes. A CT scan of the brain may not show very small tumors, so any brain scan should be performed with magnetic resonance imaging.

Treatment

Drug treatment for gelastic epilepsy includes medications that are effective in treating focal seizures, such as carbamazepine, carbamazepine such as carbamazepine, clobazam, lamotrigine, lacosamide, levetiracetam, Oxcarbazepine and topiramate. Unfortunately, none of the epilepsy drugs can stop all seizures.

Another way to treat gelastic seizures is surgery and, in rarer cases, radiotherapy, if the cause of the epilepsy is a tumor in the hypothalamus (or, less frequently, in the temporal or frontal lobes). Normally, children will need additional input at school and psychological support to help with learning problems. to help them with learning and behavioral problems.

It should be noted, however, that gelastic seizures are difficult to control. It is rare for people with this type of epilepsy to have their seizures controlled for more than a few weeks or months. Generally, the best results are seen in children and adults with gelastic epilepsy caused by a benign tumor in the hypothalamus (the hamartoma or astrocytoma) or in a temporal or frontal lobe.

Bibliographic references:

Alvarez, G. (1983). Neurología del reír patológico, a propósito de un caso de epilepsia gelástica. Rev. méd. Chile, 111(12), 1259-62. Cascino, Gregory D., F. Andermann, S. F. Berkovic, R. I. Kuzniecky, F. W. Sharbrough, D. L. Keene, P. F. Bladin, P. J. Kelly, A. Olivier, and W. Feindel. "Gelastic seizures and hypothalamic hamartomas: evaluation of patients undergoing chronic intracranial EEG monitoring and outcome of surgical treatment." Neurology 43, no. 4 (1993): 747-747. Frattali, C. M., K. Liow, G. H. Craig, L. M. Korenman, F. Makhlouf, S. Sato, L. G. Biesecker, and W. H. Theodore. "Cognitive deficits in children with gelastic seizures and hypothalamic hamartoma." Neurology 57, no. 1 (2001): 43-46.