Gelastic seizure
Encyclopedia
A gelastic seizure, also known as "gelastic epilepsy" is a rare type of seizure that involves a sudden burst of energy, usually in the form of laughing or crying. This syndrome usually occurs for no obvious reason and is uncontrollable. It is slightly more common in males than females. The term Gelastic originates from the Greek word "Gelos" which means laughter. This syndrome can go for very long periods of time without a diagnosis, as it may appear to be much like normal laughing or crying, if it occurs infrequently. It has been associated with several different conditions such as temporal
and frontal lobe
lesion
s, tumor
s, dilated temporal horns , atrophy
, tuberous sclerosis
, hemangioma
s, and post infectious foci, but mainly hypothalamic hamartomas.
are the areas of the brain with the most involvement with these seizures. This may cause learning disabilities, and faulted cognitive function as well. It is not uncommon for children to have tonic-clonic seizures, and atonic seizures directly following the seizure. Those that are associated with hypothalamic hamartomas may occur as often as several times hourly and typically begin during infancy. Seizures that occur in infancy may include bursts of cooing, respirations, giggling, and smiling. Due to early hypothalamic pituitary gonadal axis activation in girls who suffer from the seizures, it is not uncommon for them to display secondary sex characteristics before the age of eight.
is difficult, especially in children, due to the difficulty in differentiating between actual laughing or crying, versus a seizure that involves laughing and crying. In pre-verbal infants, a diagnosis may be impossible. A long history must be taken with a description of all the signs leading to and during the seizure. The episodes can also be confused with behavioral and emotional disorders. Some doctors ask parents to videotape
the children's outbursts. The tapes may be difficult to obtain, but can be very helpful in speeding up the difficult diagnosis process. Diagnosis is also complicated due to the many possible causes of the seizures. Imaging
is always helpful in an attempt to diagnose seizures caused by hypothalamic hamartoma. If there is evidence of this, the diagnosis takes much less time.
. A hypothalamic hamartoma is defined as a benign
mass of glial tissue on or near the hypothalamus
. The size of the hamartoma can vary from one centimeter to larger than three centimeters. They can cause several different types of seizures including a Gelastic Seizure. These structures can be detected with different imaging modalities such as computed tomography
and magnetic resonance imaging
. A computed tomography scan of an individual with a hypothalamic hamartoma would reveal an suprasellar mass with the same density as brain tissue. Images of these masses are not enhanced with the use of contrast. However, although a computed tomography scan may be useful in diagnosing the cause of a seizure, in the case of a hypothalamic hamartoma, magnetic resonance imaging is the tool of choice due to the cerebrospinal fluid
which defines these masses. Photon emission computed tomography may also be used. This involves the use of a radiotracer which is taken up by the ictal
region of the brain which is typically where the tumor lies.
. They can produce secondary seizure characteristics which may respond to medications or surgery
. These options are not a guaranteed cure, and depend solely on the individual patient’s pathology
.
in the United States that offer the surgical treatment. However, since not all individuals are candidates for surgery, there are other treatment options. Hormonal treatment can be attempted to help individuals with precocious puberty. Anti-epileptic drugs could be an option as well depending on the patient’s criteria. These drugs could include carbamazepine
, clobazam
, lamotrigine
, levetiracetam, oxcarbazepine
and topiramate
. However, none of these medications are capable of stopping the seizures from occurring, and like any medication, there may be undesirable side effects. There is also a specialized form of radiotherapy that may be an option depending on the tumor type and location. Once again, there are very few areas in the world that offer this treatment. Gamma knife radiosurgery
can be the treatment of choice when it comes to hypothalamic hamartomas. It is a low risk option due to its lower frequency of neurological deficits. It is recommended for patients with tumors that don’t come into contact with the optic chiasm
.
Twenty-five percent of patients who have undergone surgery have reported complications. These complications are permanent in seven percent of patients. The main difficulty reported was the loss of their short term memory. The loss typically did not last for more than three months, but in some cases it appears permanent. Researchers believe that the loss of memory may be due to injury to the language dominant hemisphere of the fornix
during surgical removal or resection
of a hypothalamic hamartoma. Resection of the hamartoma from the mamillary bodies could play a role in memory loss as well. Hemiparesis
is another complication that can occur during surgical resection, possibly due to a thalamic
perforating vessel injury. Weight gain has been reported in a few patients.
was also referred to in one paper also as "Seinfeld syncope" after an incident in which a patient repeatedly fainted while watching an episode of Seinfeld
.
Temporal lobe
The temporal lobe is a region of the cerebral cortex that is located beneath the Sylvian fissure on both cerebral hemispheres of the mammalian brain....
and frontal lobe
Frontal lobe
The frontal lobe is an area in the brain of humans and other mammals, located at the front of each cerebral hemisphere and positioned anterior to the parietal lobe and superior and anterior to the temporal lobes...
lesion
Lesion
A lesion is any abnormality in the tissue of an organism , usually caused by disease or trauma. Lesion is derived from the Latin word laesio which means injury.- Types :...
s, tumor
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
s, dilated temporal horns , atrophy
Atrophy
Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations , poor nourishment, poor circulation, loss of hormonal support, loss of nerve supply to the target organ, disuse or lack of exercise or disease intrinsic to the tissue itself...
, tuberous sclerosis
Tuberous sclerosis
Tuberous sclerosis or tuberous sclerosis complex is a rare multi-system genetic disease that causes non-malignant tumors to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin. A combination of symptoms may include seizures, developmental delay, behavioral...
, hemangioma
Hemangioma
A hemangioma of infancy is a benign self-involuting tumor of endothelial cells, the cells that line blood vessels. It usually appears during the first weeks of life and sometimes resolves by age 10. In more severe case hemangioma may have permanency, if not treated by a physician...
s, and post infectious foci, but mainly hypothalamic hamartomas.
Signs and symptoms
The main sign of a gelastic seizure is a sudden outburst of laughter or crying with no apparent cause. The laughter may sound unpleasant and sardonic rather than joyful. The outburst usually lasts for less than a minute. During or shortly after a seizure, an individual might display some twitching, strange eye movements, lip smacking, fidgeting or mumbling. If a person of who suffers from the seizures is hooked up to an electroencephalogram it will reveal interictal epileptic discharges. This syndrome usually manifests itself before the individual reaches the age of three or four. The temporal lobes, and the hypothalamusHypothalamus
The Hypothalamus is a portion of the brain that contains a number of small nuclei with a variety of functions...
are the areas of the brain with the most involvement with these seizures. This may cause learning disabilities, and faulted cognitive function as well. It is not uncommon for children to have tonic-clonic seizures, and atonic seizures directly following the seizure. Those that are associated with hypothalamic hamartomas may occur as often as several times hourly and typically begin during infancy. Seizures that occur in infancy may include bursts of cooing, respirations, giggling, and smiling. Due to early hypothalamic pituitary gonadal axis activation in girls who suffer from the seizures, it is not uncommon for them to display secondary sex characteristics before the age of eight.
Diagnosis
A diagnosisDiagnosis
Diagnosis is the identification of the nature and cause of anything. Diagnosis is used in many different disciplines with variations in the use of logics, analytics, and experience to determine the cause and effect relationships...
is difficult, especially in children, due to the difficulty in differentiating between actual laughing or crying, versus a seizure that involves laughing and crying. In pre-verbal infants, a diagnosis may be impossible. A long history must be taken with a description of all the signs leading to and during the seizure. The episodes can also be confused with behavioral and emotional disorders. Some doctors ask parents to videotape
Videotape
A videotape is a recording of images and sounds on to magnetic tape as opposed to film stock or random access digital media. Videotapes are also used for storing scientific or medical data, such as the data produced by an electrocardiogram...
the children's outbursts. The tapes may be difficult to obtain, but can be very helpful in speeding up the difficult diagnosis process. Diagnosis is also complicated due to the many possible causes of the seizures. Imaging
Medical imaging
Medical imaging is the technique and process used to create images of the human body for clinical purposes or medical science...
is always helpful in an attempt to diagnose seizures caused by hypothalamic hamartoma. If there is evidence of this, the diagnosis takes much less time.
Causes
A gelastic seizure is typically caused by a hypothalamic hamartomas, or a brain tumorBrain tumor
A brain tumor is an intracranial solid neoplasm, a tumor within the brain or the central spinal canal.Brain tumors include all tumors inside the cranium or in the central spinal canal...
. A hypothalamic hamartoma is defined as a benign
Benign
A benign tumor is a tumor that lacks the ability to metastasize. Common examples of benign tumors include moles and uterine fibroids.The term "benign" implies a mild and nonprogressive disease. Indeed, many kinds of benign tumors are harmless to human health...
mass of glial tissue on or near the hypothalamus
Hypothalamus
The Hypothalamus is a portion of the brain that contains a number of small nuclei with a variety of functions...
. The size of the hamartoma can vary from one centimeter to larger than three centimeters. They can cause several different types of seizures including a Gelastic Seizure. These structures can be detected with different imaging modalities such as computed tomography
Computed tomography
X-ray computed tomography or Computer tomography , is a medical imaging method employing tomography created by computer processing...
and magnetic resonance imaging
Magnetic resonance imaging
Magnetic resonance imaging , nuclear magnetic resonance imaging , or magnetic resonance tomography is a medical imaging technique used in radiology to visualize detailed internal structures...
. A computed tomography scan of an individual with a hypothalamic hamartoma would reveal an suprasellar mass with the same density as brain tissue. Images of these masses are not enhanced with the use of contrast. However, although a computed tomography scan may be useful in diagnosing the cause of a seizure, in the case of a hypothalamic hamartoma, magnetic resonance imaging is the tool of choice due to the cerebrospinal fluid
Cerebrospinal fluid
Cerebrospinal fluid , Liquor cerebrospinalis, is a clear, colorless, bodily fluid, that occupies the subarachnoid space and the ventricular system around and inside the brain and spinal cord...
which defines these masses. Photon emission computed tomography may also be used. This involves the use of a radiotracer which is taken up by the ictal
Ictal
Ictal refers to a physiologic state or event such as a seizure, stroke or headache. The word originates from the Latin ictus, meaning a blow or a stroke...
region of the brain which is typically where the tumor lies.
Management
Gelastic seizures are not responsive to therapyTherapy
This is a list of types of therapy .* Adventure therapy* Animal-assisted therapy* Aquatic therapy* Aromatherapy* Art and dementia* Art therapy* Authentic Movement* Behavioral therapy* Bibliotherapy* Buteyko Method* Chemotherapy...
. They can produce secondary seizure characteristics which may respond to medications or surgery
Surgery
Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
. These options are not a guaranteed cure, and depend solely on the individual patient’s pathology
Pathology
Pathology is the precise study and diagnosis of disease. The word pathology is from Ancient Greek , pathos, "feeling, suffering"; and , -logia, "the study of". Pathologization, to pathologize, refers to the process of defining a condition or behavior as pathological, e.g. pathological gambling....
.
Treatment
The treatment depends on the cause of the seizures. If the seizures are caused by a tumor, surgical removal can be attempted. However, surgical removal is not always an immediate cure, and there can be complications. Complications can include cerebral infarcts, and cognitive deterioration. There are only a few specialized centers in the United Kingdom and one in Phoenix, ArizonaPhoenix, Arizona
Phoenix is the capital, and largest city, of the U.S. state of Arizona, as well as the sixth most populated city in the United States. Phoenix is home to 1,445,632 people according to the official 2010 U.S. Census Bureau data...
in the United States that offer the surgical treatment. However, since not all individuals are candidates for surgery, there are other treatment options. Hormonal treatment can be attempted to help individuals with precocious puberty. Anti-epileptic drugs could be an option as well depending on the patient’s criteria. These drugs could include carbamazepine
Carbamazepine
Carbamazepine is an anticonvulsant and mood-stabilizing drug used primarily in the treatment of epilepsy and bipolar disorder, as well as trigeminal neuralgia...
, clobazam
Clobazam
Clobazam , is a drug which is a benzodiazepine derivative. It has been marketed as an anxiolytic since 1975 and an anticonvulsant since 1984...
, lamotrigine
Lamotrigine
Lamotrigine, marketed in the US and most of Europe as Lamictal by GlaxoSmithKline, is an anticonvulsant drug used in the treatment of epilepsy and bipolar disorder. It is also used as an adjunct in treating depression, though this is considered off-label usage...
, levetiracetam, oxcarbazepine
Oxcarbazepine
Oxcarbazepine is a anticholinergic anticonvulsant and mood stabilizing drug, used primarily in the treatment of epilepsy. It is also used to treat anxiety and mood disorders, and benign motor tics...
and topiramate
Topiramate
Topiramate is an anticonvulsant drug. It was originally produced by Ortho-McNeil Neurologics and Noramco, Inc., both divisions of the Johnson & Johnson Corporation. This medication was discovered in 1979 by Bruce E. Maryanoff and Joseph F. Gardocki during their research work at McNeil...
. However, none of these medications are capable of stopping the seizures from occurring, and like any medication, there may be undesirable side effects. There is also a specialized form of radiotherapy that may be an option depending on the tumor type and location. Once again, there are very few areas in the world that offer this treatment. Gamma knife radiosurgery
Radiosurgery
Radiosurgery is a medical procedure that allows non-invasive treatment of benign and malignant tumors. It is also known as stereotactic radiotherapy, when used to target lesions in the brain, and stereotactic body radiotherapy when used to target lesions in the body...
can be the treatment of choice when it comes to hypothalamic hamartomas. It is a low risk option due to its lower frequency of neurological deficits. It is recommended for patients with tumors that don’t come into contact with the optic chiasm
Optic chiasm
The optic chiasm or optic chiasma is the part of the brain where the optic nerves partially cross...
.
Surgical complications
Twenty-five percent of patients who have undergone surgery have reported complications. These complications are permanent in seven percent of patients. The main difficulty reported was the loss of their short term memory. The loss typically did not last for more than three months, but in some cases it appears permanent. Researchers believe that the loss of memory may be due to injury to the language dominant hemisphere of the fornix
Fornix
The fornix is a C-shaped bundle of fibers in the brain, and carries signals from the hippocampus to the hypothalamus.-Structure:...
during surgical removal or resection
Segmental resection
Segmental resection is a surgical procedure to remove part of an organ or gland. It may also be used to remove a tumor and normal tissue around it. In lung cancer surgery, segmental resection refers to removing a section of a lobe of the lung.- External links :* entry in the public domain NCI...
of a hypothalamic hamartoma. Resection of the hamartoma from the mamillary bodies could play a role in memory loss as well. Hemiparesis
Hemiparesis
Hemiparesis is weakness on one side of the body. It is less severe than hemiplegia - the total paralysis of the arm, leg, and trunk on one side of the body. Thus, the patient can move the impaired side of his body, but with reduced muscular strength....
is another complication that can occur during surgical resection, possibly due to a thalamic
Thalamus
The thalamus is a midline paired symmetrical structure within the brains of vertebrates, including humans. It is situated between the cerebral cortex and midbrain, both in terms of location and neurological connections...
perforating vessel injury. Weight gain has been reported in a few patients.
Outlook
Like many other types of seizures, gelastic seizures are hard to control for an extended period of time. The best outlook is for children suffering the seizures due to a benign tumor in their hypothalamus. The removal of these tumors can be effective not only for the frequency of the seizures, but also the behavioral and cognitive symptoms that come along with the syndrome.Media references
Gelastic syncopeSyncope (medicine)
Syncope , the medical term for fainting, is precisely defined as a transient loss of consciousness and postural tone characterized by rapid onset, short duration, and spontaneous recovery due to global cerebral hypoperfusion that most often results from hypotension.Many forms of syncope are...
was also referred to in one paper also as "Seinfeld syncope" after an incident in which a patient repeatedly fainted while watching an episode of Seinfeld
Seinfeld
Seinfeld is an American television sitcom that originally aired on NBC from July 5, 1989, to May 14, 1998, lasting nine seasons, and is now in syndication. It was created by Larry David and Jerry Seinfeld, the latter starring as a fictionalized version of himself...
.