A seizure is an abnormal electrical discharge from the brain. It may affect a small focal area of the brain, or the entire brain (generalized). The area affected by the seizure loses its regular ability of function and may react uncontrollably. For example, if an area of the brain that controls an arm has a seizure, the arm may shake repetitively. If a seizure affects the entire brain, all the extremities may shake uncontrollably. Some seizures may present with staring and unresponsiveness. Theoretically, any function of the brain, motor, smell, vision, or emotion may be individually affected by a seizure. The seizure, however, for the most part will always follow the same pattern in a given individual.
Epilepsy or a seizure disorder (same meaning) is defined by having 2 or more seizures. The seizures must be of an unprovoked cause. Meaning that there is no immediate connectable cause for the seizures, such as low blood glucose, exposure to toxins, alcohol withdraw, immediate effect of a trauma, of fever in young children (less than 6 years of age).
Seizures are generally divided into 2 main types:
Focal seizures may spread to the rest of the brain, therefore becoming focal seizures with secondary generalization.
Febrile seizures are convulsive events that are considered benign (not associated with serious difficulties), occurring between 6 months and 6 years of age. The typical febrile seizure is a convulsive event that lasts about one to five minutes. This usually occurs with the rapid rise of the fever and consists of a rhythmic jerking of the extremities, eye rolling, unresponsiveness, sometimes cyanosis (bluish discoloration around the mouth and the tips of the extremities), followed by 30 minutes of drowsiness and confusion. As the temperature normalizes, the child may return to his normal self. An occasion, a febrile seizure may occur differently, non-convulsive (without shaking), presenting a loss of tone and consciousness or with stiffening of the body.
Some children may have complex febrile seizures. Complex features include the following:
The significance of the complex features is that of a higher risk for future epilepsy; the more complex the features, the higher the risk for epilepsy or seizures without fever. Generally if a child has a simple febrile seizure the risk for epilepsy is 2% compared to 1% in the general population. The risk for future febrile seizures is about 30%, or 50% if the first seizure occurred before one year of age. Also, the lower the temperature that provoked the first febrile seizure is, the higher the risk for future febrile seizure events.
Treatment for febrile seizures is usually unnecessary. Lowering of the temperature with Tylenol or Motrin is usually ineffective. Anticonvulsants may be used in unusual situations, usually continuous Phenobarbithal or Depakene (Depakene after 3 years of age). Oral valium as premedication, given intermittently during febrile illnesses is highly effective and does not require continuous medication administration and monitoring. This is my first choice for febrile seizures that require treatment.
Febrile seizures are not considered to cause any damage to the brain. Studies have shown no difference in intelligence between children who suffer from febrile seizures or their siblings (or identical twins) who do not have febrile seizures. Some recent studies even suggest improved memory function in children who have had febrile seizures.
Complications from febrile seizures are rare and are mostly associated with focal and prolonged febrile seizures. Future epilepsy, especially with recurrent focal seizures, was reported. Respiratory compromise is rare and may be caused by prolonged convulsions affecting the respiratory muscles and breathing.
Mesial temporal sclerosis, or scarring of the inner part of the temporal lobe, called the hippocampus, is a condition thought-to-be-caused by recurrent focal febrile seizures. The hippocampus, if damaged, is highly epileptogenic (causing seizures). If mesial temporal sclerosis develops, this is associated with a form of epilepsy (partial complex seizures), difficult to control. This, however, is a rare and questionable complication of febrile seizures.
In an individual who suffers from seizures, one of the most important things to avoid is drowning. Drowning is one of the most common causes of death from Epilepsy. It is very important to ensure that a person with seizures will not be left alone in the water; (bathtub, Jacuzzi or pool) without supervision. Also people with Epilepsy should not go on a small boat without a partner in order to avoid falling overboard during a seizure and drowning.
The other thing to avoid is unnecessary head trauma. A person with Epilepsy should try to avoid engaging in sports that predispose one to repeated head injury (such as boxing). The question is less clear when it comes to other sports. In principle, one would like to lead a regular (as normal as possible) life style, not to be crippled incessantly by the disease. To date, there are no clear guidelines of the American Academy of Neurology that pertain to each sport specifically such as football, soccer and basketball. What I generally suggest is to use common sense and avoid head trauma without creating unnecessary hardships. This involves judgment that has to take into account the severity of Epilepsy.
Other precautions involve avoiding situations that predispose one to a higher risk of seizures, such as staying up late and disrupting the structured daily routine, (circadian rhythm). One must slow the level of security when an intercurrent illness or fever are present and control fever with Tylenol (acetaminophen). Some parents make the mistake of stopping the antiepileptic drugs when Tylenol and antibiotics are given for fever. Unfortunately, this is the riskiest time for seizures, and antiepileptic drug administration must be continuous and uninterrupted.
Some people are sensitive to flickering lights, strobe lights, and other photic stimulations. A person with epilepsy should avoid such situations as disco light exposure or sitting on the sunny side of a car when trees block the light intermittently and create an intermittent photic stimulation.
Eating well, sleeping well, and using common sense is potentially life saving and could allow a person with Epilepsy to live a full, happy life without unreasonable of excessive limitations.
The first most important rule for any stressful situation is DO NOT PANIC!! Keep yourself as calm as possible under the circumstances. A seizure is a scary sight, especially if it is prolonged. As in any medical emergency, the first step is the ABC’s of emergencies. (A), for airway, (B) for breathing and (C), for circulation.
In a case of a seizure the airway is the main concern. Protecting the airway is the most important action an observer can take. Positioning the individual on his or her side, leaning the head against the forearm so that the face and the mouth point downwards, this will enable gravity to clear the oral secretions and push the tongue outwards. This action is sufficient in most circumstances to protect the patient from harm during the duration of the epileptic convulsion. Do not attempt to prop the mouth open, pull out the tongue, or place the person on his back during the convulsion. All these actions may cause further bleeding into the airway or cause regurgitation or secretion/tongue obstructing the airway. In most instances, the seizure will be over in 1-5 minutes. That is when the position may be adjusted, and the situation reassessed. Call 911 as soon as possible if a high degree of concern exists. Children who have frequent daily short seizures, however, whose parents are familiar with the situation do not require a hospital visit for each seizure. In these circumstances, the parents must exercise common sense but should not take any unnecessary risks.
In a situation where breathing has stopped, on extremely rare occasions, (B), Breathing assistance may be required. Parents of children with a known tendency to stop breathing should be trained in CPR and have an ambo-bag as well as an oxygen tank handy at home. (C), Circulatory assistance is hardly ever required and is reserved for the hospital emergency department.
Measures to be taken during a seizure include: administration of Diastat, rectal Valium gel that helps stop the seizure within a few minutes. For those who have an implanted VNS (Vagal Nerve Stimulator), swiping the magnet will deliver an immediate electrical impulse that may shorten the seizure significantly as well. Following a seizure, the caregiver must assess the situation. If in doubt, call 911 or your physician in order to plan the next step, which includes future superior protection against further recurrent seizures. This may include remembering to administer the medications (anticonvulsants), as prescribed, making sure that the child keeps the medicine down, shaking the bottle of liquid medications (especially Dilantin, which tends to sediment at the bottom on the container), going to sleep on time, increasing the dose of the medications, or changing the medication regimen for better seizure control. Compliance with the prescribed medication regimen and keeping your physician informed of any mishaps is the best recipe for good seizure control. If poor seizure control is present for an extended period of time, obtaining a second opinion may be helpful.
The causes for seizures include the immediate causes for acute seizures and the chronic causes for epilepsy or a seizure disorder. The acute causes include hypoglycemia (low blood sugar, hypocalcemia (low blood calcium), meningitis, bacterial toxins (such as shigella), alcohol withdrawal, environmental toxins, electrical shock, and side effects of medication. Penicillin overdose may also cause a seizure. Chronic causes for epilepsy include genetic epilepsy (benign rolandic, absence and juvenile myoclonic epilepsies are some examples), congenital brain malformation associated with some neurocutaneous disorders (tuberous sclerosis, neurofibromatosis), migrational defects (where gray matter migrates to the wrong brain region during early development. Other causes include chronic effects of trauma or infection that did cause brain damage or damaged an area called the hippocampus in the front central temporal lobe that if damaged, becomes highly epileptogenic.
If a child had a single definite seizure and all of the above evaluations are normal the risk for further seizures will be about 25%. This doesn’t justify treatment unless the seizure was life threatening.
If the EEG is abnormal, the risk increases to a minimum of 65-70% for further seizures in the next 4 years. This does justify treatment. Each of the other evaluations done above have similar contributions to the risk. Treatment must be considered based on the risk of leaving a child untreated.
Since seizures are sudden conditions that usually occur “out of the blue” without any warning signs, any other condition that is sudden and is associated with a change in the level of consciousness or control over function may mimic seizures.
In the pediatric (children) age group, these conditions may be divided to the age of the child:
There is, however, a simple treatment. In several studies, kids were shown to respond to iron supplementation even when no clear anemia was demonstrated. This simple treatment should be considered I children who have frequent breath holding spells.
This age group may still experience late onset breath holding spells and migraine variants as above.
Other conditions include the following:
The decision to treat children for seizures has to be a responsible serious decision based on the statistical risk for further seizures. Treatment is generally continued for two (2) years after the last seizure.
In those who’ve had one (1) seizure event, have normal development, a normal neurological examination, and a normal EEG and MRI, the risk for further seizures is about 25% for the next four (4) years. In this situation, no treatment will be initiated. If any of the above turn out to show an abnormality indicating an increased seizure risk or if a second seizure has occurred, treatment with anticonvulsant medications will be started for two (2) years.
During treatment blood tests for liver functions, blood counts must be obtained on regular intervals.
Development of anti-epileptic medications was “very slow” until 1997, when the first new anti-epileptic drug was introduced. Prior to this, the traditional anti-epileptic medications were the only option.
Good for generalized and partial seizures, and may be used for febrile seizures. Recently, more frequently used in the neonatal period and rarely used after 5 years of age because of its potential to cause learning difficulties. Side effects include hyperactivity and other behavioral difficulties in about half of the children treated (ages 2-10). Drowsiness, rashes, and learning difficulties may also occur. Rare impairments of liver functions and blood counts were reported as well. Phenobarbital is best tolerated in children less than 1 year of age.
Same as Phenobarbital. Metabolizes to Phenobarbital plus PEMA. It causes more sedation than Phenobarbital and may be helpful in some seizures poorly controlled with Phenobarbital.
Most effective in partial seizures, but also good for generalized seizures and status epilepticus. Dilantin is an excellent medication for and emergency situation, such as status epilepticus (a prolonged seizure lasting more than 20 minutes. It is also helpful for the control of seizures that occur on an intermittent basis. The problem with phenitoin, however, is that it is plagued by many side effects including hypertrophy (swelling) of the gums, coursing of facial features, hair growth on facial areas, and brain atrophy over a long period of use. It may also cause the regular side effects, including allergic reaction, and blood count and liver enzyme dysfunction. The allergic reaction may be severe (Steven Johnson’s) and life threatening.
Tegretol in its various forms is an excellent medication. It is mostly used for partial seizures. Myoclonic seizures and atypical absence seizure may worsen significantly due to Tegretol. In general it is very well tolerated, has some transient sedation, usually resolving in 3 days. Tegratol may also cause life threatening blood count and liver dysfunction. It may also cause a severe rash of Steven Johnson’s typed and other allergic rashes.
Valproic acid is an excellent anti-convulsant for generalized, as well as focal seizures. Recently it has started to gain use for non-convulsive disorders including migraine prevention and as a mood stabilizer for manic-depressive disorders.
Valproic acid comes in depakene and depakote forms. The depakote is better tolerated with regards to abdominal pain syndromes due to an enteric coating protection.
Seizures controlled with this medication include myoclonic seizures, absence seizures, and mixed type seizures. Side effects of valponic acid include initial transient sedational and abdominal pain (better tolerated with the depakote form). It may also increase the appetite, causing weight gain, cause some transient hair loss that improves with zinc supplementation, and rarely cause liver and blood count abnormalities. Hyperammonemia and pancreatic dysfunction were reported as well. In young children younger than 3, and especially younger than 2, valponic acid may cause a severe fatal liver disease in a frequency as high as 1 per 300.
Many patients taking valponic acid may have an associated carnitine deficiency. Carnitine can be supplemented with carnitor syrup or tablets.
Good for absence seizures only. Zarowtin is one of the safest anti-convulsant available. Hematological side effectsand allergic reactions were reported. Drowsiness, headaches, and abdominal pains may occur as well.
Klonopin is a benzodiazepine (valium like medication). It may be helpful for myoclonic generalized and partial seizures. It may help for infantile spasms or Lenox-Gastaut syndrome. Side effects are mostly related to sedation, drooling, cognitive impairment, and hyperactivity.
Since the early 1990’s, 9 new anti-epileptic medications were introduced to the market. These are, for the most part, medications designed to work on a specific seizure control mechanism and, therefore, at times more safe and effective than the old anti-convulsants.
A very effective anti-convulsant, even for severe and resistant seizures, such as Lenox-Gastaut syndrome, generalized absence, myoclonic and focal seizures.
Now its use is limited to extremely rare instances due to severe fatal blood and liver damage associated with this medication.
Trilepital is an excellent and very safe anti-convulsant. It is very similar to caramazepine (Tegretol) in structure, but designed to have the epoxide moiety. The epoxide is the part of carbamazepine responsible for the drowsiness, the severe allergic reactions and the very concerning liver damage and blood dyscrasia that may cause fatal side effects. The seizure control abstained with Trilepital is as good and in some situations better than with Tegretol. Side effects are minor, may cause some temporary drowsiness, mild allergic reaction, and decreased sodium level in the elderly. In short, Trilepital is an improved Tegretol, more effective, better tolerated and that has never been the cause of any fatal side effects.
Another excellent anti-epileptic medication with multiple mechanisms of action. Good for focal and generalized seizures, was found helpful with Lenox-Gastaut syndrome, infantile spasm, and other seizure types. Generally it is very well tolerated. It may cause a decrease in the appetite and psychosis. At times it may also be associated with a temporary sedative effect. For some children it may be the only medication to be able to completely control frequent intractable seizures.
This is another anti-convulsant medication with a design in mind. Gabapentin was an attempt to mimic the GABA molecule. GABA is an inhibitory neurotransmitter; theoretically increasing GABA concentration would decrease the brain excitation and stop seizures. Taking GABA itself (available in health food stores) would be useless, since GABA doesn’t cross the blood-brain barrier; Neurontin was designed to have a GABA structure yet be able to penetrate into the brain. It is a very safe anti-convulsant, but its efficacy is questionable. To achieve good seizure control, one has to treat patients with very high doses of Neurontin. The side effects include mostly some dizziness and drowsiness.
Other uses were found for Neurontin, including the treatment of migraines (as a preventative agent), neuropathies, and trigeminal neuralgia. The actual mechanism of action of this medication is unknown, but it clearly isn’t what it was designed to be.
Its main indication is as an adjunct therapy for partial seizures.
Good for generalized and partial seizures, may be effective for absence seizures, atomic seizures, and Lenox-Gastaut syndrome.
It is an effective well tolerated medication that may be associated with the development of a severe rash, especially if combined with valproic acid. Recent studies, however, indicate that the rash doesn’t occur frequently if the dose is very gradually titrated upwards. Other side effects are mild and consist mostly of some dizziness, drowsiness, or headaches.
Unavailable in the United States due to some irreversible changes in visual fields reported in association with its use. Still a very important anti-epileptic medication due to its beneficial effect in infantile spasms. It was shown to stop these severe seizures in 3 out of 4 children who have touberous sclerosis and infantile spasms. It is associated with much less adverse reaction compared to ACTH (the standard treatment for infantile spasms).
Incidentally, of my patients, the ones who were treated with Sabril for the infantile spasms had the best long-term outcome. All of them currently seizure-free, off any other anti-convulsants, and followed by me for some mild AD/HD and behavioral difficulties or some mild speech delay.
Sabril is another one of the GABA designer drugs and the only designer drug that is actually and effectively doing what it was designed to do. Sabril blocks the degradation of GABA, by blocking the effect of the enzyme GABA transamiase, thus increasing GABA concentration in the presynaptic area.
Other side effects of Sabril, other than the visual field abnormalities include rare psychosis and some other mild fatigue or gastrointestinal upset, which are related.
This is the third GABA designer medication. It blocks the reuptake of GABA in the synaptic area to be metabolized, therefore increasing GABA concentration, reducing brain excitation. It is mostly used as an adjunct for partial seizures, but may be effective in other seizure types as well. Generally it is well tolerated and was never reported to cause serious side effects. Unfortunately it has a poor effect on infantile spasm treatment and cannot be a substitute to Sabril as previously hoped.
An effective adjunct medication for partial seizure control, this one has no serious side effects reported. It currently has limited experience in the United States and has not been approved for children yet. For resistant partial seizures however, this medication should be attempted before resorting to invasive managements.
It come in 100mg capsules and is effective for the treatment of both partial and generalized seizures. It is very effective in absence seizure control and may substitute the use of Depakote in some of the absence seizures resistant to Zarontin or in those who have both absence and generalized convulsive seizures. Generally well tolerated, but in some children is causing extreme drowsiness. Zonegran is usually well tolerated in adults and may cause some rare but serious hematological side effects.
In most situations, the treatment of a seizure disorder lasts for about 2 years. Standard of care suggests treatment for two years after the last seizure. At that point, if the EEG normalizes and there are no brain lesions (detectable on MRI, CT, or physical examination) that may predispose an individual for further seizures, the risk for recurrent seizure events will be as low as 25-30%, which doesn’t require further treatment. If however the EEG stays abnormal the risk for further recurrent events after 2 years of treatment rises to about 50% or more. Under this circumstance, treatment will be continued for at least 1 year.
If a recurrent seizure occurred after 2 years of being seizure free under the effect of anti-epileptic medication, treatment should be resumed for another 2 years. This applies to seizures that are relatively easy to control.
For seizures refractory to the treatment of a single anti-convulsant and those who have multiple recurrent seizure events, treatment for epilepsy may be a life-long requirement. Alternative seizure managements may be an option for those who continue to have frequent seizures, despite the use of different anticonvulsants.
Most children will present with a single seizure and a normal EEG recording. These will not require treatment, just an evaluation that includes a neuro-developmental assessment, an EEG and a MRI of the brain. About 75% of those who have a normal evaluation (as above) will never have another seizure and will continue to lead a normal life. Still, depending on the severity of the seizure and its duration, one may make an exception, especially if dealing with a single seizure that is life threatening in nature. Parents of these children may be prescribed Diastat, a rectal injector containing Valium in a gel form. This may stop a seizure within 3 minutes and continue to protect the child for up to 8 hours from further seizures, which in most situations is a sufficient amount of time to get medical attention.
For children who present with a single seizure, but have abnormal EEG, or for those who have a normal EEG, but a second seizure, the standard of care suggests treatment. The best treatment consists of a single agent, Tegretol (in a slow release form, if possible), Depakote, or Carbatrol, preferably not Dilantin for a prolonged duration of treatment, due to the adverse reactions. Phenobarbital should also be avoided if possible in children older than 2, due to the hyperactivity and learning impairments associated with its use.
Once a medication has been chosen, treatment should start gradually, with a low therapeutic level initially. If seizures recur, the level may be gradually increased until no further seizures occur. If seizures become worse as the medication level is increased, it should be substituted. If the level is high therapeutic and seizures continue, another medication should be used. Whether as a substitute or as an adjunct is dependent on the amount of benefit obtained from the first medication and other considerations (such as the EEG pattern).
More than 2 anti-epileptic medications may be used in combination to attain the best seizure control. This will cover the needs of the majority of the patients. For those 5-10% whose seizures still remain intractable, other options are available, including the Ketogenic diet, epilepsy surgery, and vagus nerve stimulation.
For the most part, the anti-convulsant medications are well tolerated. A serious concern includes the rare (yet possible) potential for the development of a fatal abnormality if the blood cells. Tegretol may affect the white blood cell count, causing an immune deficiency, or affect all the blood cells causing a rare yet fatal aplastic anemia. Dilantin and Depakene may cause similar, but more infrequent difficulties. Depakene also causes a dose related platelet deficiency. The liver may be affected by all the traditional anti-convulsant, but most seriously in children younger than 3 years of age. A frequent 1 in 300 liver disorder may be associated with Depakene use. Allergic reactions may occur, less commonly with Tegretol, a severe Steven Johnson’s reaction may occur. This rash is potentially fatal and may require intensive care unit hospitalization.
The newer anti-convulsants are generally safer except for Felbatol and may cause some specific side effects causing discomfort for the most part.
For specific side effects of the different anticonvulsants, please refer to What are Anti-Epileptic Medications?
Seizures, if left untreated, are associated with a high incidence of injury and may be associated with fatality as well. Treatment with anti-convulsant medications prevents these complications. Other seizures, if left untreated may severely impair the quality of life, such as absence (staring) seizures and some short partial-complex seizures. In adults, other issues, such as driving are of extreme importance.
Even when treated, occasional breakthrough seizures may occur. This is important to remember, because drowning is a common cause of death from epilepsy. For example, people have had seizures while fishing on their own and fallen overboard, there have also been cases of drowning in the bath due to convulsions. The lifestyle of an epileptic should be logically adjusted to prevent such occurrences.
This is a difficult diet to maintain, but it does prevent seizures, especially in younger children (1-8 years). Seizures that fail to be controlled with anti-convulsant medications may respond well to this diet.
The diet’s mechanism is based on the principle that starvation may prevent seizures. During a starvation period, fat is broken down for energy metabolism. When fat is broken, ketone bodies are formed. These are acetone, acetoacetic acid, and beta-aminobutric acid. The ketone bodies have anti-seizure properties. By applying the Ketogenic diet, one stimulates a starvation situation without actively starving. This is accomplished by feeding an individual only fat. When fat is consumed it is broken into the ketone bodies as if these are the actual fat stores of the body. During the period of treatment (usually 2 years), a child cannot have any carbohydrate intake (bread, pasta, sugar, or flour products). Proteins are limited to a small amount and the entire diet is strictly calculated to the gram. Also the fluid intake is limited in order to maintain a proper concentration of ketones.
The general notion is that the diet stops seizures in 30%, reduces them in 30%, and is ineffective in about 30%. The best candidates are children who have frequent small generalized or focal seizures who didn’t respond to any anticonvulsant or can’t tolerate them due to side effects and are between 1 and 8 years of age. Some severe seizure forms such as Lenox-Gastaut syndrome or infantile spasms may respond well to the diet.
The diet is not free of side effects, other than the inconvenience of maintaining it. The diet may cause growth retardation, kidney stones, side effects related to exposure to fat, and more. Proper vitamin supplementation, calcium, and mineral supplementation must be maintained while on the diet. Still, with all its difficulties, for the right candidate with a motivated capable family, this is an excellent option to be considered.
In vagus nerve stimulation, a coil is wrapped around the vagus nerve and is stimulated electrically with a pacemaker-like device that is placed under the skin, below the clavicle. The repetitive stimulation of the vagus nerve delivers an impulse to the brain that is protective against seizures. The implementation of the device requires a minor surgical procedure.
The device becomes activated about 2 weeks following the implementation and the neurologist controls the amount of stimulation delivered. Other than seizure protection, vagus nerve stimulation is reported to improve memory, decrease side effects, as these are being weaned off. Recently, some reports indicate beneficial effects in mood disorders.
Most commonly consists of resectioning an epileptogenic (seizure producing) brain region. Surgery is usually the last resort for patients who fail to respond to other anti-epileptic managements. There are several surgical options depending on the type and location of the seizure focus.
The most successful epilepsy surgery is for the treatment of partial seizures when a clear lesion can be detected, such as in the case of mesial temporal sclerosis, or the atrophy of the gray matter in a very specific area at the tip of the temporal lobe. When the lesion is on the right side, chances are better that language will not be affected by surgery. Prior to the surgery, however, close EEG monitoring is required in order to localize the seizure focus precisely and to prevent the extraction of functional brain regions. In some situations an EEG grid is implanted close to the brain in order to obtain more accurate monitoring.
The results of surgery are generally good, making it a valid option for the appropriate candidate. Families, however, should be aware of some potential surgical complications, the possibility (depending on the seizure type) that seizures will continue after the surgery, and that medication may be needed in combination with surgery to obtain the best seizure control.