Gelstic epilepsy: symptoms, causes and treatment

We all know the beneficial effects of laughter. Laughing helps us relax and feel better, and it can even ease pain. However, it's not always like that.

In the movie "Joker", starring Joaquin Phoenix, we can see how his character, Arthur Fleck, shows a laugh sudden and strident that you seem unable to control and that causes you to have real problems living and communicating with the rest.

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

Throughout the article we will talk about gelastic epilepsy, its causes, the symptoms it causes, and how to diagnose and treat this disorder.

• Related article: "Epilepsy: definition, causes, diagnosis and treatment"

What is gelastic epilepsy?

Gelstic 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 gellastic crises. This disease is slightly more common in boys than girls, although it is 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 lead to gelastic seizures are the hypothalamus (a region with an important role in various autonomic and endocrine visceral 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 a astrocytoma.

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

Most of these tumors are benign. This means that they can 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, older children who have gelastic epilepsy caused by a hypothalamic hamartoma also commonly have learning and behavior problems They tend to get worse in the middle or late teens.

• You may be interested: "Parts of the human brain (and functions)"

Symptoms

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

Laughter occurs suddenly, appears for no obvious reason, and is usually completely out of place. Older children can sometimes predict 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 by the name of "aura", a peculiar sensation that warns of the proximity of epileptic seizures.

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 back and forth movements of the eyes and the head, automatisms (such as lip smacking, murmuring or moving hands), altered consciousness or lack of response to people who surround. These signs can last from seconds to many minutes and then stop.

Gellastic seizures can occur along with other types of seizures, either immediately after or at other times. These include tonic-clonic seizures, which occur with stiffness throughout the body and jerking of the arms and legs with loss of consciousness; and atonic seizures, in which there is a weakness of all the muscles of the body.

Diagnosis

When making a diagnosis in gelastic epilepsy a detailed history and description of the child's seizures should be sought. Laughter episodes can be mistaken for behavioral or emotional disorders, even in children with attention difficulties or characteristics of autism spectrum disorder. Therefore, the latter can delay diagnosis and is more likely to occur in young children.

Sometimes a sound video of the child's episodes can be very helpful in ruling out or confirming the disorder. Nevertheless, an electroencephalogram is necessary to detect focal and generalized abnormalities (sharp waves, spikes or spikes, and slow waves).

Likewise, for a correct diagnosis it is also necessary to perform a brain scan to determine the existence or not of tumors located in the hypothalamus or in other brain areas, such as the temporal lobes or frontal. A brain CT scan may not show very small tumors, so any brain scan should be done with MRI.

Treatment

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

Another way to stop gelastic seizures is surgery and, in more rare cases, radiation therapy, in case the cause of the epilepsy is a tumor in the hypothalamus (or, less commonly in the temporal lobes or frontal). Usually, children will need additional input at school and psychological support to help them with learning and behavior problems.

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

Bibliographic references:

Alvarez, G. (1983). Neurology of pathological laughing, about a case of gelastic epilepsy. Rev. med. 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.