We all know the beneficial effects of laughter. Laughing helps us relax and feel better, and can even relieve 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 sudden and strident laugh that he seems unable to control and that makes him have real problems living and communicating with others.
The disease that underlies the uncontrolled laughter of the film’s protagonist is none other than gelastic epilepsy, a very rare type of epilepsy that is characterized by the manifestation of epileptic seizures in the form of laughter.
Throughout the article we will talk about gelastic epilepsy its causes, the symptoms it causes, and how to diagnose and treat this disorder.
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 crises. 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 several 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 an 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 the 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 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.
Additionally, it is common for older children who have gelastic epilepsy caused by a hypothalamic hamartoma to also have learning and behavioral problems which usually worsen in middle or late adolescence.
Symptoms
Gelastic epilepsy seizures can begin at any age, but usually do so before age 3 or 4. Seizures usually begin with a laugh that is often described as “hollow” or “empty” and not very pleasant, although sometimes it may 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 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 attacks.
Laughter usually lasts 30 to 45 seconds and stops suddenly It may then be followed by signs more frequently seen in focal seizures, such as back and forth movements of the eyes and head, automatisms (such as smacking the lips, mumbling, or moving the hands), alterations in consciousness, or the lack of response to the people around them. These signs can last from seconds to many minutes and then stop.
gelastic seizures They can manifest together with other types of seizures, either immediately afterward or at other times. These include tonic-clonic seizures, which cause stiffness throughout the body and jerking of the arms and legs with loss of consciousness; and atonic seizures, in which weakness occurs in all the muscles of the body.
Diagnosis
When making a diagnosis in gelastic epilepsy a detailed history and a description of the seizures the child has suffered should be inquired Laughter episodes can be confused with behavioral or emotional disorders, even in children with attention difficulties or features of autism spectrum disorder. Therefore, the latter can 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. Nevertheless, it is necessary to perform an electroencephalogram 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 or frontal lobes. A CT scan of the brain may not show very small tumors, so any brain scan must be done with magnetic resonance imaging.
Treatment
Pharmacological 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 tackle gelastic seizures is surgery and, in more rare cases, radiation therapy, if the cause of the epilepsy is a tumor in the hypothalamus (or, less frequently in the temporal or frontal lobes). Usually, Children will need additional contributions at school and psychological support to help them with learning and behavioral 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 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 (a hamartoma or astrocytoma) or in a temporal or frontal lobe.
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