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

In the film “Joker”, starring Joaquin Phoenix, we can see how his character, Arthur Fleck, shows a sudden and strident laughter that he can’t seem to control and that makes him have real problems to live and communicate with others.

The disease underlying the protagonist’s uncontrolled laughter is none other than gelatinous epilepsy, a very rare type of epilepsy characterised by the manifestation of epileptic seizures in the form of laughter.

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

What is gelatinous epilepsy?

Gelatinous epilepsy is a type of epilepsy in which “gelatinous” seizures occur. This term comes from the Greek word “gelastikos” which means laugh in our language, so it is precisely this inappropriate laugh which manifests itself in gelptic 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 result in gel seizures are the hypothalamus (a region with a major 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 may be one of two types: a hamartoma or an astrocytoma.

A hamartoma is a benign (noncancerous) growth made up of an abnormal mixture of cells and tissues 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 (supporting cells of the nervous system).

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

In addition, it is common for older children who have gelatinous epilepsy caused by a hypothalamic hamartoma to also have learning and behavioral problems that usually worsen in the mid to late teens.

Symptoms

The seizures of gelatinous epilepsy can begin at any age, but usually before age 3 or 4. The seizures usually begin with a laugh that is often described as “hollow” or “empty” and not very pleasant, although it may sometimes sound like a child’s normal laugh.

Laughter occurs suddenly, appears for no obvious reason, and is usually completely out of place. Sometimes older children can 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 often in focal crises, such as movements back and forth of the eyes and head, automatisms (such as lip smacking, mumbling or hand movements), altered consciousness or lack of response to people around them. These signs may last from seconds to many minutes and then stop.

Jelly-like seizures may occur along with other types of seizures , either immediately after or at other times. These include tonic-clonic seizures, which involve stiffness throughout the body and shaking of the arms and legs with loss of consciousness; and atonic seizures, in which all the muscles in the body are weakened.

Diagnosis

When making a diagnosis of epilepsy in gelatine , a detailed history and description of the seizures the child has suffered should be sought . Laughing episodes may be confused with behavioral or emotional disorders, even in children with attention difficulties or characteristics of autism spectrum disorder. Therefore, the latter may delay diagnosis and is more likely to occur in young children.

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

For a correct diagnosis, a brain scan is also needed 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 scan of the brain must be done with MRI.

Treatment

Drug treatment for gel 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 medicines can stop all seizures.

Another way to stop gel seizures is surgery and, in more rare cases, radiation therapy, if a tumor in the hypothalamus (or, less often, the temporal or frontal lobes) is the cause of the epilepsy. Normally, children will need extra input at school and psychological support to help them with learning and behavioural problems.

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

Bibliographic references:

Alvarez, G. (1983). Neurology of pathological laughter, regarding a case of gelatinous epilepsy. 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 y W. H. Theodore. “Déficit cognitivo en niños con convulsiones gelásticas y hamartoma hipotalámico”. Neurología 57, no. 1 (2001): 43-46.