What Is Gelastic Epilepsy?
Gellastic epilepsy is known as that convulsive pathology whose main symptom is the uncontrollable and involuntary laughter of the person suffering from it. It is not a known disease, except now for those interested in the movie Joker, released recently. In it, the plot plays with the possibility that the protagonist suffers from it.
As such, gelastic epilepsy has been diagnosed since 1957, when scientists Daly and Mulder published a research paper that describes it. The article appeared in the journal Neurology and laid the foundations for the description of the clinical picture.
The term ‘gelastic’ has its origin in a Greek word that we could translate as ‘laughter ‘ . Certainly, the symptom par excellence that distinguishes this epilepsy from others is the laugh that appears spontaneously, even in situations where it is not justified at all.
This uncontrollable and involuntary laughter does not come from a joke or a situation that warrants it. Moreover, many patients remember the episode of laughter with sadness, associating distant feelings with joy or pleasure.
This confirms that the pathology is uncomfortable and is not a mere laughing episode. Patients feel that what is happening is inappropriate, but suffer from not being able to put a limit on it. In addition, it is impossible for them to prevent the appearance of laughter.
Gellastic epilepsy is more common in men than in women. Even so, its incidence is very low, approximately 0.2%; This means that for every thousand people there may be two who have the disease. Its classic age of onset is between three and four years old.
Causes of gelastic epilepsy
Gellastic epilepsy can be an atypical form of seizure that responds to little-known genetic mechanisms or mechanisms of neuronal connections. In any case, there is an underlying cause that has been associated and it is the hypothalamus tumor.
Two types of tumors can develop in the hypothalamus: hamartomas and astrocytomas. Although these are tumors that tend to be benign, their slow but sustained growth presses on structures generating different symptoms.
As benign tumors, both hamartomas and astrocytomas do not metastasize. Nor do they invade neighboring organs to the hypothalamus.
Differential diagnostics
The laughter of gelastic epilepsy poses a diagnostic challenge for clinicians. This inappropriate and uncontrollable laughter is not necessarily always a form of seizure. It can also be due to other pathologies.
Before a drugged or alcoholic person, it is possible to find a laugh similar to that of gelastic epilepsy. Obviously, it is not a symptom that appears chronic, but rather triggers with consumption. That is the key point to differentiate one entity from the other.
There is also a syndrome known as Angelman that has laughter as a symptom. It is a disabling genetic alteration. In Angelman syndrome there is a marked developmental delay. From six months, parents can notice that their child has a problem with his nervous system.
Finally, the entity that causes the most confusion is pseudobulbar palsy. Possibly, the point of differentiation between this and gelastic epilepsy is that, in pseudobulbar palsy, laughter appears in people who suffer from another underlying disorder, such as Parkinson’s disease, for example.
Symptoms of gelastic epilepsy
We have already said it and we repeat it: the primary symptom of gelastic epilepsy is laughter. Now, it is not a laugh like any other, but it has special characteristics that make it unique and different.
The laughter of gelastic epilepsy lasts less than a minute and appears, as well as disappears, suddenly. It is involuntary and the patient cannot decide to stop it because he has no control over it.
Along with laughter, classic seizure symptoms sometimes appear, such as movements through muscle spasms, momentary loss of consciousness, and sphincter relaxation. These symptoms can last even when the laughter is over.
In children who start with gellastic laughter and develop signs of precocious puberty, it is very likely that there is a hamartoma growing in the hypothalamus. Precocious puberty is understood as the appearance of signs of adolescence before the age of eight or nine.
Treatment
Gellastic epilepsy is treated with the same medications that are used for seizures. Among them, we can mention carbamazepine, lamotrigine, topiramate or levetiracetam, among others.
Laughter symptom control is not easy to achieve. Unfortunately, the treatment is never effective enough. Laughter crises recur cyclically, profoundly affecting the quality of life of patients.
Despite the difficulties involved, it is important to know that much progress has been made in anti-epileptic drug research. In addition, today, many groups of professionals in the world are more educated in the disease and more trained to deal with it.
