Gelastic epilepsy: symptoms, causes and treatment

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

In the movie “Joker”, starring Joaquin Phoenix, we see how his character, Arthur Fleck, shows a sudden, shrill laugh that seems out of control and really makes it hard for him to live and communicate with others.

The underlying disease under 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 epilepsyAmong its causes, 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 “gelásticas” convulsions 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 in 1,000 children with this brain disorder.

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

    the 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: 1 hamartoma or astrocytoma.

    A hamartoma is a benign (non-cancerous) growth made up of an abnormal mixture of cells and tissues that is usually found in the area of ​​the body where the growth takes place. An astrocytoma is a tumor of the nervous system that develops 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, he is more likely to be detected 1:00 hypothalamic hamartoma.

    In addition, it is common for older children who have gelastic epilepsy caused by hypothalamic hamartoma to also have learning and behavior problems which usually get worse in the middle to late adolescence.

      symptoms

      Gelastic epileptic seizures can start at any age, but usually before 3 or 4 years old. Seizures usually start with laughter often described as “empty” or “empty” and not very pleasant, although it can sometimes sound like normal laughter in a child.

      Laughter comes on suddenly, comes on 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 happen. These sensations have also been described in other types of epilepsy and are called “aura,” a particular sensation that warns of the proximity of epileptic seizures.

      Laughter usually lasts 30 to 45 seconds and stops suddenly. It can then be followed by signs more often observed during focal seizures, such as side-to-side movements of the eyes and head, automatisms (such as bursting lips, mumbling or moving hands), alterations in consciousness. or lack of response to those around them. These signs can last from a few seconds to several minutes and then stop.

      Gelastic crises they can appear with other types of seizures, Either immediately afterwards or at other times. These include tonic-clonic seizures, which run stiffly throughout the body and tremors of the arms and legs with loss of consciousness; and atonic seizures, in which weakness occurs in all the muscles of the body.

      diagnostic

      When diagnosing gelastic epilepsy it must be studied in the detailed history and in a description of the seizures to which the child has suffered. Laughter episodes can be mistaken for behavioral or emotional disturbances, even in children with attention difficulties or features of autism spectrum disorders. Therefore, the latter can delay diagnosis and is more likely to occur in young children.

      Sometimes a video with the sound of the child’s episodes can be very helpful in ruling out or confirming the disorder. however, an electroencephalogram should be performed to detect focal and generalized anomalies (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 areas of the brain, such as the temporal or frontal lobes. CT scans of the brain may not show very small tumors, so any scan of the brain should be done using MRI.

      treatment

      Pharmacological treatment for gelastic epilepsy includes effective drugs to treat focal seizures such as carbamazepine, clobazam, lamotrigine, lacosamide, levetiracetam, oxcarbazepine and topiramate. Unfortunately, none of the medicines for epilepsy can stop all seizures.

      Another way to end gelastic seizures is surgery and, in rarer cases, radiation therapy, in case the cause of epilepsy is a tumor in the hypothalamus (or, less often in the temporal lobes or front).). normally children will need additional school contributions and psychological support to help them solve 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. Usually, the best results are seen in children and adults with gelastic epilepsy caused by a benign tumor of the hypothalamus (hamartoma or astrocytoma) or of a temporal or frontal lobe.

      Bibliographical references:

      Alvarez, G. (1983). Neurology of pathological laughter, in a case of gelastic epilepsy. Tower. med. Chile, 111 (12), 1259-62. Cascino, Gregory D., F. Andermann, SF Berkovic, RI Kuzniecky, FW Sharbrough, DL Keene, PF Bladin, PJ 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. Fractals, CM, K. Liow, GH Craig, LM Korenman, F. Makhlouf, S. Sato, LG Biesecker, and WH Theodore. “Cognitive Deficits in Children with Gelastic Seizures and Hypothalamic Hamartoma”. Neurology 57, no. 1 (2001): 43-46.

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