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Epilepsy

Highlights

What Is Epilepsy?

Epilepsy is a brain disorder that is marked by recurrent seizures. Seizures (also called fits or convulsions) are episodes of disturbed brain function caused by abnormalities of the brain’s electrical activity. There are many different types of seizures.

Causes

Epilepsy can affect people of all ages but is most common in young children and older adults. Some types of epilepsy are inherited and due to genetic factors. Other possible causes of epilepsy include brain injuries such as head trauma or oxygen deprivation at birth. In many cases, the cause of epilepsy is unknown (idiopathic).

Diagnosis

A doctor will diagnose epilepsy based on a patient’s medical history, description of seizures, and various diagnostic tools. The most important diagnostic tool is the electroencephalogram (EEG), which allows doctors to record and analyze brain waves. Imaging tests such as computed tomography (CT) and magnetic resonance imaging (MRI) may also be used.

Treatment

The goal of epilepsy treatment is to control seizures. Many different types of anticonvulsant drugs are available to treat epilepsy. Some patients need only one drug, while others may need to take several drugs. For patients who have not been helped by medication, surgery or a neurostimulation device may be an option. Dietary changes, such as the ketogenic diet, have shown promise in helping some patients, especially children with severe epilepsy.

Anti-epileptic drugs (AEDs) can cause many side effects. Pregnant women with epilepsy need to take special precautions, because some of these drugs (particularly valproate) can cause birth defects.

AEDs and Birth Defects

In recent years the FDA has issued several warnings on birth defect risks and AEDs:

  • Valproate  may have long-term effects on a child’s cognitive development if the mother takes it during her first trimester of pregnancy. Studies indicate that children born to mothers who took valproate during pregnancy had lower scores on IQ and other cognitive tests than children whose mothers took other types of AEDs. Valproate drug products include valproate sodium (Depacon), valproic acid (Depakene), and divalproex sodium (Depakote).
  • Topiramate  (Topamax, generic) may increase the risk for cleft palate or cleft lip when taken during the first trimester of pregnancy.

FDA Approves New Neurostimulation Device

For nearly 20 years, vagus nerve stimulation (VNS) was the only FDA-approved brain stimulation (neurostimulation) treatment for epilepsy. This changed in 2013, when the FDA approved a new type of neurostimulator device called the RNS stimulator. RNS stands for responsive neurostimulation.

This “brain pacemaker” is designed to detect electrical signals indicating an impending seizure, and send electrical stimulation to stop the seizure before it occurs. The FDA has approved the device for adults with partial onset seizures that have not responded to medication. Like the VNS, the RNS is surgically implanted.

Introduction

Epilepsy is a brain disorder involving repeated, spontaneous seizures of any type. There are different types of epilepsy but what they all share are recurrent seizures caused by an uncontrolled electrical discharge from nerve cells in the cerebral cortex. This part of the brain controls higher mental functions, general movement and sensation, the functions of the internal organs in the abdominal cavity, perception, and behavioral reactions.

Brain structure
The structures of the brain include the brainstem, consisting of the spinal cord, the medulla oblongata, the pons and the midbrain; the cerebellum; the cerebrum (one half, or hemisphere shown); and the diencephalon.

Seizures

Seizures are a symptom of epilepsy. Seizures ("fits," convulsions) are episodes of disturbed brain function that cause changes in neuromuscular function, attention, or behavior. They are caused by abnormally excited electrical signals in the brain.

Seizures can also be caused by conditions other than epilepsy. Many people experience a single seizure at some point in their life and then never experience another. A single seizure may be related to a specific medical problem (such as fever, a reaction to a drug, or withdrawal from alcohol) This is different from epilepsy.

Epilepsy is a condition that causes permanent changes in the brain and disturbs how electrical energy works in the brain. The seizures that are associated with epilepsy are provoked by abnormal bursts of electrical activity in the brain.

Types of Epilepsy

Epilepsy is generally classified into two main categories based on seizure type:

  • Partial (also called focal or localized) seizures. Partial seizures are more common than generalized seizures and occur in one or more specific locations in the brain. In some cases, partial seizures can spread to wide regions of the brain. They are likely to develop from specific injuries, but in most cases the exact origins are unknown (idiopathic).
  • Generalized seizures. Generalized seizures typically occur in both sides of the brain. Many forms of these seizures are genetically based. The patient usually has normal neurologic function between episodes.

Partial Seizures (also called Focal Seizures)

Partial (focal)  seizures are subcategorized as "simple" or "complex partial":

  • Simple Partial Seizures. A person with a simple partial seizure (sometimes known as Jacksonian epilepsy) does not lose consciousness, but may experience confusion, jerking movements, tingling, or odd mental and emotional events. Such events may include deja vu, mild hallucinations, or extreme responses to smell and taste. After the seizure, the patient usually has temporary weakness in certain muscles. These seizures typically last about 90 seconds.
  • Complex Partial Seizures. Slightly over half of all seizures in adults are complex partial type. About 80% of these seizures originate in the temporal lobe, the part of the brain located close to the ear. Temporal lobe disturbances can result in loss of judgment, involuntary or uncontrolled behavior, or loss of consciousness. Patients may lose consciousness briefly and appear to others as motionless with a vacant stare. Emotions can be exaggerated; some patients even appear to be drunk. After a few seconds, a patient may begin to perform repetitive movements, such as chewing or smacking of lips. Episodes usually last no more than 2 minutes. They may occur infrequently, or as often as every day. A throbbing headache may follow a complex partial seizure.

In some cases, simple or complex partial seizures evolve into what are known as secondarily generalized seizures. The progression may be so rapid that the initial partial seizure is not even noticed.

Generalized Seizures

Generalized seizures are caused by nerve cell disturbances that occur in more widespread areas of the brain than partial seizures. Therefore, they have a more serious effect on the patient. They are further subcategorized as tonic-clonic (or grand mal), absence (petit mal), myoclonic, or atonic seizures.

Tonic-Clonic (Grand Mal) Seizures. The first stage of a grand mal seizure is called the tonic phase, in which the muscles suddenly contract, causing the patient to fall and lie stiffly for about 10 - 30 seconds. Some people experience a premonition or aura before a grand mal seizure. Most, however, lose consciousness without warning. If the throat or larynx is affected, there may be a high-pitched musical sound (stridor) when the patient inhales. Spasms occur for about 30 seconds to 1 minute. Then the seizure enters the second phase, called the clonic phase. The muscles begin to alternate between relaxation and rigidity. After this phase, the patient may lose bowel or urinary control. The seizure usually lasts a total of 2 - 3 minutes, after which the patient remains unconscious for a while and then awakens to confusion and extreme fatigue. A severe throbbing headache similar to migraine may also follow the tonic-clonic phases.

Absence (Petit Mal) Seizures. Absence or petit mal seizures are brief losses of consciousness that occur for 3 - 30 seconds. Physical activity and loss of attention may pause for only a moment. Such seizures may pass unnoticed by others. Young children may simply appear to be staring or walking distractedly. Petit mal may be confused with simple or complex partial seizures, or even with attention deficit hyperactivity disorder (ADHD). In petit mal, however, a person may experience attacks as often as 50 - 100 times a day.

Myoclonic. Myoclonic seizures are a series of brief jerky contractions of specific muscle groups, such as the face or trunk.

Atonic (Akinetic) Seizures. A person who has an atonic (or akinetic) seizure loses muscle tone. Sometimes it may affect only one part of the body so that, for instance, the jaw slackens and the head drops. At other times, the whole body may lose muscle tone, and the person can suddenly fall. A brief atonic episode is known as a drop attack.

Simply Tonic or Clonic Seizures. Seizures can also be simply tonic or clonic. In tonic seizures, the muscles contract and consciousness is altered for about 10 seconds, but the seizures do not progress to the clonic or jerking phase. Clonic seizures, which are very rare, occur primarily in young children, who experience spasms of the muscles but not tonic rigidity.



Click the icon to see an image of a grand mal seizure.

Epilepsy Syndromes

Epilepsy is also grouped according to a set of common characteristics, including:

  • Patient age
  • Type of seizure or seizures
  • Behavior during seizure
  • Results of EEG recordings
  • Whether a cause is known or not known (idiopathic)

A few syndromes and inherited epilepsies are listed below; they do not represent all epilepsies.

Temporal Lobe Epilepsy. Temporal lobe epilepsy is a form of partial (focal) epilepsy, although generalized tonic-clonic seizures may occur with it.

Frontal Lobe Epilepsy. Frontal lobe epilepsy is characterized by sudden violent seizures. Seizures may also produce loss of muscle function, including the ability to talk. In autosomal dominant nocturnal frontal lobe epilepsy, a rare inherited form, seizures often occur during sleep.

West Syndrome (Infantile Spasms). West syndrome, also called infantile spasms, is a disorder that involves spasms and developmental delay in children within the first year, usually in infants ages 4 - 8 months.

Benign Familial Neonatal Convulsions. Benign familial neonatal convulsions (BFNC) are a rare, inherited form of generalized seizures that occur in infancy. BFNC appears to be caused by genetic defects that affect channels in nerve cells that carry potassium.

Juvenile Myoclonic Epilepsy (Impulsive Petit Mal). Juvenile myoclonic epilepsy, also called impulsive petit mal epilepsy, is characterized by generalized seizures, usually tonic-clonic marked by jerky movements (called myoclonic jerks), and sometimes absence seizures. It usually occurs in younger people ages 8 - 20.

Lennox-Gastaut Syndrome. Lennox-Gastaut syndrome is a severe form of epilepsy in young children that causes multiple seizures and some developmental disability. It usually involves absence, tonic, and partial seizures.

Myoclonic-Astatic Epilepsy. Myoclonic-astatic epilepsy (MAE) is a combination of myoclonic seizures and astasia (a decrease or loss of muscular coordination), often resulting in the inability to sit or stand without aid.

Progressive Myoclonic Epilepsy. Progressive myoclonic epilepsy is a rare inherited disorder typically occurring in children ages 6 - 15. It usually involves tonic-clonic seizures and marked sensitivity to light flashes.

Landau-Kleffner Syndrome. Landau-Kleffner syndrome is a rare epileptic condition that typically affects children ages 3 - 7. It results in the loss of ability to communicate either with speech or by writing (aphasia).

Seizures That Are Not Epilepsy

Nonepileptic Seizures. Nonepileptic seizures refer to events that resemble epileptic seizures but aren’t caused by abnormal electrical activity in the brain. Nonepileptic seizures may be physical or psychological in origin.

Physiologic nonepileptic seizures result from conditions such as sudden drops in blood pressure (syncope), abnormal heart rhythms (cardiac arrhythmia), low blood sugar associated with diabetes (hypoglycemia), or sleep disorders such as narcolepsy. Psychogenic nonepileptic seizures are usually due to emotional trauma or stress.

Febrile Seizures. Febrile seizures are convulsions in children triggered by a high fever. Most febrile seizures occur in young children ages 9 months to 5 years. Simple febrile seizures last for less than 15 minutes and only occur once in a 24-hour period. They are usually an isolated event and not a sign of underlying epilepsy. However, complex febrile seizures, which last longer than 15 minutes and occur more than once in 24 hours, may be a sign of underlying neurologic problems or epilepsy.

Eclampsia. Eclampsia is a life-threatening pregnancy complication associated with tonic-clonic seizures. It can result in pregnant women who develop preeclampsia, a condition marked by extremely high blood pressure and excess fluid in the lungs.

First Seizure. As noted above, a single seizure may be due to causes other than epilepsy (fever, infections, drug reactions, head trauma). Many people who have a first seizure will never have another seizure, and do not have epilepsy. Still, a doctor will usually order a brain imaging test to make sure that epilepsy is not an underlying cause.

Causes

Epileptic seizures are triggered by abnormalities in the brain that cause a group of nerve cells in the cerebral cortex (gray matter) to become activated simultaneously, emitting sudden and excessive bursts of electrical energy. A seizure's effect depends in part on the location in the brain where this electrical hyperactivity occurs. Effects range from brief moments of confusion to minor spasms to loss of consciousness. In most cases of epilepsy, the cause is unknown (idiopathic).

Nerve conduction

 Click the icon to see an animation detailing epilepsy. 

Brain Chemistry Factors

Ion Channels. Sodium, potassium, and calcium act as ions in the brain. They produce electric charges that must fire regularly in order for a steady current to pass from one nerve cell in the brain to another. If the ion channels that carry them are genetically damaged, a chemical imbalance occurs. This can cause nerve signals to misfire, leading to seizures.

Neurotransmitters. Abnormalities may occur in neurotransmitters, the chemicals that act as messengers between nerve cells. Three neurotransmitters are of particular importance: Scientists in epilepsy research have paid particular attention to the neurotransmitter gamma aminobutyric acid (GABA), which helps prevent nerve cells from over-firing.

Genetic Factors

Some types of epilepsy are inherited conditions where genetics play a factor. Generalized epilepsy seizure types appear to be more related to genetic influences than partial seizure epilepsies.

Brain Damage

Epilepsy can be caused by many types of diseases or injuries that damage the brain. Head trauma can bring on seizures and epilepsy. Cerebral palsy and other disorders caused by lack of oxygen to the brain during birth are often associated with epilepsy.

Epilepsy can also develop from another medical condition that affects the brain or central nervous system. Examples include:

  • Brain tumors
  • Traumatic brain injury, stroke, or a transient ischemic attack (TIA)
  • Infections in the central nervous system (brain abscess, meningitis, encephalitis, neurosyphilis, AIDS)
  • Poisoning caused by prolonged exposure to certain types of chemicals (lead, carbon monoxide)

Risk Factors

Age

Epilepsy affects all age groups but is most common in the very young and the very old. The risk is highest in children under the age of 2 and in older adults over age 65. In infants and toddlers, prenatal factors and birth delivery problems are associated with epilepsy risk.

In children age 10 and younger, generalized seizures are more common. In older children, partial seizures are more common.

Gender

Males have a slightly higher risk than females of developing epilepsy.

Family History

People who have a family history of epilepsy are at increased risk of developing the condition.

Complications

People with epilepsy generally have a normal lifespan. However, they are at risk for health complications.

Accident-Related Injuries

Injuries from Falls. Because many people with seizures fall, injuries are common. Although such injuries are usually minor, people with epilepsy have a higher incidence of fractures than those without the disorder. Certain medications (phenytoin, carbamazepine) can affect bone hardness or density and increase the risk for fractures.  .

Household Accidents. Household environments, such as the kitchen and bathroom, can be dangerous places for children with epilepsy. Parents should take precautions to prevent burning accidents from stoves and other heat sources. Children with epilepsy should never be left alone when bathing.

Driving. Due to the high risk for accidents, people with epilepsy who have seizures that are not controlled by medication are legally restricted from driving. In general, to obtain a driver's license, a doctor must confirm that a patient has been seizure-free for –a specific number of months.

Swimming. People with epilepsy have a much higher risk for drowning than people without this condition. In fact, one study suggested the drowning risk for people with epilepsy is 15 – 19 times greater than for the general population. People with epilepsy who swim should avoid deep and cloudy water (a clear swimming pool is best), and always swim with a knowledgeable, competent, and experienced companion or at a facility that has a lifeguard on site.

Mental Health Complications

Depression and anxiety are common among people with epilepsy. People with epilepsy have a higher risk for suicide, particularly in the first 6 months following diagnosis. The risk for suicide is highest among people who have epilepsy and an accompanying psychiatric condition, such as depression, anxiety disorder, schizophrenia, or chronic alcohol use. All antiepileptic drugs can increase the risk of suicidal thoughts and behavior. [For more information, see Medications section in this report.].

Learning and Memory Problems

Children with epilepsy often experience problems with learning, attention focus, and memory retention. Some of these problems are due to the after-effects of seizures. Medication side effects, including fatigue and drowsiness, can also be contributing factors. Problems with learning and memory can affect school performance, and lead to behavioral issues. .

Sexual and Reproductive Health Complications

Effects on Sexual Function. Some patients with epilepsy experience sexual dysfunction, including erectile dysfunction. These problems may be caused by emotional factors, medication side effects, or a result of changes in hormone levels.

Effects on Reproductive Health. A woman’s hormonal fluctuation can affect the course of her seizures. Estrogen appears to increase seizure activity, and progesterone reduces it. Antiseizure medications may reduce the effectiveness of oral contraceptives.

Pregnancy Complications. Epilepsy can pose risks both to a pregnant woman and her fetus. Some types of anti-epileptic drugs should not be taken during the first trimester because they can cause birth defects. Women with epilepsy who are thinking of becoming pregnant should talk to their doctors in advance to plan changes in their medication regimen. Women should learn about the risks associated with epilepsy and pregnancy, and precautions that can be taken to reduce them. [For more information, see “Treatment during Pregnancy” in Treatment section of this report.]

Status Epilepticus

Status epilepticus (SE) is a prolonged seizure that is a serious, potentially life-threatening condition. It is a medical emergency. Permanent brain damage or death can result if the seizure is not treated effectively.

The condition is generally defined as a continuous seizure that lasts for more than 5 minutes. There are two forms of status epilepticus: generalized convulsive, which involve prolonged seizures, and non-convulsive, which affects behavior and consciousness. About 25% of people who experience status epilepticus have pre-existing epilepsy. Most times, this condition occurs in people who do not have epilepsy and who have never had a prior seizure.

In people with epilepsy, status epilepticus is usually caused by failure to take anticonvulsant medications as directed, or by the abrupt withdrawal from certain medications. Other general causes of status epilepticus include alcohol intoxication or withdrawal, infections, fever, metabolic disorders, stroke, brain tumors, and head trauma.

SUDEP

Although relatively rare, there is a risk for sudden unexpected death in patients with epilepsy, a syndrome abbreviated as SUDEP. The causes of such events are not fully known, but heart arrhythmias and pauses in breathing (apnea) may be factors in many cases. Your doctor can explain if you have specific risk factors for SUDEP and what protective measures can be taken.

The best preventive measure is to take your medication as prescribed. Talk with your doctor if you have any concerns about medication side effects or dosages. Do not make any changes to your drug regimen without speaking first with your doctor.

Prognosis

The prognosis for patients with epilepsy depends on various factors. In general, patients whose epilepsy is well-controlled by medications have an excellent prognosis and many experience short- and even long-term remission from seizures.

The outlook is best for patients whose epilepsy responds to treatment soon after being diagnosed. The longer a patient remains free from seizures, the lower the chance of seizure recurrence. Some patients are able to reduce or even stop their anti-seizure medications after having no seizures for several years.

Patients who tend to have a poorer prognosis include those with chronic, active epilepsy (high frequency of seizures) that does not respond to early treatment. Patients who have other illnesses in addition to epilepsy (diabetes, heart disease) are also at increased risk for developing other health problems.

Certain types of childhood epilepsy go away or improve with age, usually by the late teens or 20s. The outlook for childhood epilepsy depends in part on the specific epilepsy syndrome. Syndromes such as childhood absence epilepsy have excellent prognosis, with many children outgrowing the syndrome and experiencing remission by their teenage years. Other syndromes, such as juvenile myoclonic epilepsy, can be well-controlled by medication but are likely to be lifelong.

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Diagnosis

An epilepsy diagnosis is often made during an emergency visit for a seizure. If a person seeks medical help for a previous or suspected seizure, the doctor will ask about the patient's medical history, including seizure events.

Diagnostic Tools

Electroencephalogram (EEG). The most important diagnostic tool for epilepsy is an EEG, which records and measures brain waves, which reflect the electrical activity of nerve cells in the brain. Ideally, it should be performed within 24 hours of a seizure. An EEG recording session may last for less than an hour, but in some cases the doctor will want a day-long recording or a recording during sleep. Long-term monitoring may be necessary when patients do not respond to medications. Portable EEG units may be available, which can be used to monitor patients throughout normal activities. EEGs are not foolproof. Repeated EEGs are often needed to confirm a diagnosis, particularly for certain partial seizures that often produce an initially normal EEG reading.

Video Electroencephalography (Video EEG). For this test, patients are admitted to a special part of the hospital where they are monitored both by EEG and are also watched by a video camera. Patients may need to undergo video EEG monitoring for a variety of reasons including withdrawal or addition of medications in a patient with difficult-to treat-epilepsy, before epilepsy surgery for some patients, and also when nonepileptic seizures are suspected.

Computerized Tomography (CT) Scans. A CT scan is usually the initial brain imaging test ordered for most adults and children with first-time seizures. This imaging technique is sensitive enough for most purposes. In children, even if the scan is normal, the doctor will follow up to be sure other problems are not present.

CT scan of the brain
A CT (computed tomography) scan is a much more sensitive imaging technique than x-ray, allowing high definition of both the bony structures and the soft tissues. Clear images of organs such as the brain, muscles, joint structures, veins and arteries, as well as anomalies like tumors and hemorrhages may be obtained with or without the injection of contrasting dye.

Magnetic Resonance Imaging (MRI). Doctors strongly recommend MRIs for children with first seizures who are younger than age 1 year or who have seizures that are associated with any unexplained significant mental or motor problems. MRI scans may help to determine if the disorder can be treated with surgery, and may be used as a guide for surgeons.

Other Advanced Imaging Techniques. Some research centers use other types of imaging techniques. Positron emission tomography (PET) may help locate damaged or scarred locations in the brain where partial seizures are triggered. These findings may help determine which patients with severe epilepsy are good candidates for surgery. Single-photon emission computer tomography (SPECT) may also be used to decide if the surgery should be performed and what part of the brain needs to be removed. Both of these imaging techniques are generally only needed when an MRI of the brain has not been helpful.

Ruling Out Other Conditions

Conditions that may cause symptoms similar to epilepsy include:

  • Syncope. Syncope (fainting), a brief lapse of consciousness, in which blood flow to the brain is temporarily reduced, can mimic epilepsy. It is often misdiagnosed as a seizure. Patients with syncope do not have the rhythmic contracting and then relaxing of the body's muscles.
  • Migraines. Migraine headaches, particularly migraine with auras, may sometimes be confused with seizures. With epileptic seizure, the preceding aura is often seen as multiple, brightly colored, circular spots, while migraine sufferers tend to see black, white, or colorless lined or zigzag flickering patterns. Typically the migraine pain expands gradually over minutes to encompass one side of the head.
  • Panic Attacks. In some patients, partial seizures may resemble panic attacks, which are a type of anxiety disorder. Symptoms of a panic attack include palpitations, sweating, trembling, sensation of breathlessness, chest pain, feeling of choking, nausea, faintness, chills or flushes, fear of losing control, and fear of dying.
  • Narcolepsy. Narcolepsy, a sleep disorder that causes a sudden loss of muscle tone and excessive daytime sleepiness, can be confused with epilepsy.

Treatment

What To Do When Someone Has a Seizure

You cannot stop a seizure, but you can help the patient prevent serious injury.

Remain calm, and do not panic, then take the following actions:

  • Wipe away any excess saliva to prevent obstruction of the airway. Do not put anything in the patient's mouth. It is not true that people having seizures will swallow their tongues.
  • Turn the patient gently on the side. Do not try to hold the patient down to prevent shaking.
  • Rest the patient's head on something flat and soft to protect it from banging on the floor and to support the neck.
  • Move sharp objects out of the way to prevent injury.

Do not leave the patient alone. Someone nearby should call 911. Patients should be taken to an emergency room when:

  • A first-time seizure occurs
  • Any seizure lasts beyond 2 - 3 minutes
  • The patient has been injured
  • The patient is pregnant
  • The patient has diabetes
  • Parents, caregivers, or bystanders are at all uncertain

Not all patients with chronic epilepsy need to go to the hospital after a seizure. Hospitalization may not be necessary for patients whose seizures are not severe or repetitive, and who have no risk factors for complications. All patients or caregivers, however, should contact their doctors after a seizure occurs.

Starting Drug Treatment

Treatment with anti-epileptic drugs (AEDs) is usually initiated or strongly considered for the following patients:

  • Children and adults who have had two or three seizures. (If there was either a long period of time between seizures or the seizure was provoked by an injury or other specific causes, the doctor may wait before starting AEDs. In children, risk for recurrence after a single unprovoked seizure is rare. The risk after a second seizure is also low, even when the seizure is prolonged.)
  • Children and adults after a single seizure if tests (EEG or MRI) reveal any brain injury, or if specific neurologic, developmental, or epilepsy syndromes put a person at special risk for recurrence, for instance, in cases of myoclonic epilepsy.

There is some debate about whether to treat every adult patient with an AED after a single initial seizure. Some doctors do not recommend treating adult patients after a single seizure if they have a normal neurologic examination, EEG, and imaging studies.

Determining an Anti-Epileptic Drug (AED) Regimen

Epileptic seizures can often be controlled using a single-drug regimen. First-line anti-epileptic drugs include phenytoin (Dilantin, generic), carbamazepine (Tegretol, generic), and divalproex sodium (Depakote, generic). Patients generally begin with low doses and build up to higher doses until the seizures are controlled or a toxic reaction occurs. If a single drug fails to control seizures, other drugs are added on. The specific drugs and whether more than one should be used are determined by various factors, including the patient's age and the seizure type, frequency, and cause.

Monitoring Effects

During the first few months of therapy, the doctor will probably order blood tests once or twice to monitor drug levels and, if necessary, adjust dosages. Monitoring is used to check for drug side effects and complications, and to be sure the patient is complying with the regimen. These blood tests may be, however, a less reliable indicator of problems than patients’ own observations of response to the drug. For instance, blood tests may suggest that the dosage levels are insufficient according to general standards, yet the individual patient may be seizure-free and leading a normal life.

Anti-epileptic drugs interact with many other drugs, and may cause special problems in older patients who use multiple medications for other health problems. Elderly patients should have liver and kidney function tests performed before starting antiseizure medication. It is also very important that women have AED levels monitored during pregnancy (see "Treatment During Pregnancy" below).

Discontinuing Drug Therapy

Most patients who have responded well to medications can stop taking AEDs within 5 - 10 years after last experiencing a seizure. Evidence suggests that medications in children should not be halted for at least 2 years after the last seizure, particularly if they have partial seizures and abnormal EEGs. It is not clear whether children who have been free of generalized seizures need to wait more than 2 years or if they can withdraw earlier.

Treatment During Pregnancy

Preparing to Become Pregnant. Women with epilepsy who are considering pregnancy should talk to their doctors before they become pregnant. According to guidelines from the American Academy of Neurology and the American Epilepsy Society:

  • A woman who has been seizure-free for at least 9 months before becoming pregnant has a good chance of remaining seizure free during pregnancy.
  • Folic acid is recommended for all pregnant women. Women with epilepsy should talk with their doctors about taking folic acid supplements at least 3 months before conception as well as during the pregnancy.
  • Women with epilepsy do not face a substantially increased risk of premature birth or labor and delivery complications (including cesarean section). However, women with epilepsy who smoke may face a higher risk of premature delivery than women without epilepsy who smoke.
  • Babies born to mothers who use an AED during pregnancy may be at increased risk of being small for their gestational age.

Medication Use During Pregnancy. Women should discuss with their doctors the risks of anti-epileptic drugs (AEDs) and the possibility of making any changes in their drug regimen in terms of dosages or prescriptions. According to current guidelines:

  • Women with epilepsy should consider taking only one AED during their pregnancy to reduce the risk of birth defects. Taking more than one AED may increase birth defect risk. Carbamazepine is generally considered to be the safest AED for use during pregnancy.
  • Of all AEDs, valproate carries the highest risk for birth defects and should be avoided, if possible, during the first trimester. Valproate use has been associated with neural tube defects, facial clefts (cleft lip or palate), and hypospadias (abnormal position of urinary opening on the penis). Valproate may cause poor cognitive outcomes in children exposed to it in the womb. In studies, children born to mothers who took valproate during pregnancy had lower scores on IQ and other cognitive tests than children whose mothers took other types of AEDs. Some research  suggests valproate may increase the risk for autism and autism spectrum disorders. Women who take valproate during childbearing years should be sure to use effective birth control.
  • Phenytoin and phenobarbital may also cause cognitive impairment.
  • Topiramate (Topamax, generic) may increase the risk for cleft lip or palate birth defects when taken during the first trimester of pregnancy.
  • Pregnancy can affect how an AED is metabolized in your body. AED levels tend to drop during pregnancy. Your doctor should monitor your blood levels throughout your pregnancy to see if your dosage needs to be adjusted. This is especially important if you take lamotrigine, carbamazepine, or phenytoin.

Breastfeeding. If women on AEDs breastfeed they should be aware that some types of AEDs are more likely than others to pass into the breast milk. The following AEDs appear to be the most likely to pass into breast milk in clinically important amounts: Primidone, levetiracetam, and possibly gabapentin, lamotrigine, and topiramate. Valproate does pass into breast milk, but it is unclear if it affects the nursing infant. A mother should watch for signs of lethargy or extreme sleepiness in her infant, which could be caused by her medication. Talk with your doctor about any concerns you have about breastfeeding and AEDs.

Medications

Anti-epileptic drugs (AEDs) include many types of medications but all act as anticonvulsants. Many newer AEDs are better tolerated than the older, standard AEDs, although they can still have troublesome side effects. Newer AEDs often cause less sedation and require less monitoring than older drugs. Although newer AEDs are generally FDA-approved for use as add-ons to standard drugs that have failed to control seizures, they are often prescribed as single drugs. Specific choices usually depend on the patient’s particular condition and the specific side effects of the AED.

All antiepileptic drugs can increase the risks of suicidal thoughts and behavior (suicidality). Research has shown that the highest risk of suicide can occur as soon as 1 week after beginning drug treatment and can continue for at least 24 weeks. Patients who take these drugs should be monitored for signs of depression, changes in behavior, or suicidality.

There are dozens of anti-epileptic drugs. The following are some of the most commonly prescribed.

Valproate

Valproate sodium (Depacon, generic), valproic acid (Depakene, generic), and  divalproex sodium (Depakote, generic) are anticonvulsants that are chemically very similar to each other. (In this report, they are collectively referred to as valproate.) Valproate products are the most widely prescribed anti-epileptic drugs worldwide. They are the first choice for patients with generalized seizures and are used to prevent nearly all other major seizures as well.

Side Effects. These drugs have a number of side effects that vary depending on dosage and duration. Most side effects occur early in therapy and then subside. The most common side effects are upset stomach and weight gain. Less common side effects include dizziness, hair thinning and loss, and difficulty concentrating.

Serious side effects include:

  • A higher risk for serious birth defects than other AEDs especially if taken during the first trimester of pregnancy. In particular, these drugs are associated with facial cleft deformities (cleft lip or palate) and cognitive impairment.  [See "Treatment During Pregnancy" in Treatment section.]
  • Liver damage or failure is a rare but extremely dangerous side effect that usually affects children under 2 years of age who have birth defects and are taking more than one antiseizure drug.
  • Pancreatitis (inflammation of the pancreas) and kidney problems are also rare but serious side effects.

Carbamazepine

Carbamazepine (Tegretol, Equetro, Carbatrol, and generic) is used for many types of epilepsy including partial seizures, generalized tonic-clonic (grand mal) seizures, and mixed seizures. A chewable form is available for children.

Side Effects. Common side effects of carbamazepine include dizziness, drowsiness, problems with walking and coordination, nausea, and vomiting.

Carbamazepine can make hormonal forms of birth control less effective. It can also interact with many types of prescription medication.

More serious side effects may include:

  • Rare but life-threatening skin reactions, including toxic epidermal necrolysis and Stevens-Johnson syndrome. These skin reactions cause rash, mouth sores, peeling and blistering skin, and other severe symptoms. People of Asian ancestry have a 10 times greater risk for skin reactions than other ethnicities.
  • Bone marrow blood disorders such as aplastic anemia and agranulocytosis.
  • Patients should be sure to inform their doctors if they have any sign of sore throat, fever, easy bruising, or unusual bleeding, or severe fatigue,
  • Long-term therapy may cause bone density loss (osteoporosis) in women.
  • Birth defects can occur if taken during pregnancy. This drug may also be passed through breast milk.

Note: Grapefruit, Seville oranges, and tangelos can increase carbamazepine's blood levels and risk of adverse effects.

Phenytoin

Phenytoin (Dilantin, generic) is often prescribed as a first-line drug to treat generalized tonic-clonic (grand mal) seizures and complex partial seizures. This drug may be used alone or in combination with other AEDs.

Side Effects. The most common side effects of phenytoin include problems with walking and coordination, slurred speech, confusion, dizziness, trouble sleeping, and tremor.

More serious side effects may include:

  • Softening of the bones (osteomalacia), which can lead to fractures;
  • Severe and rare life-threatening skin reactions (Stevens-Johnson syndrome, toxic epidermal necrosis)
  • Birth defects if taken during pregnancy
  • Drug interactions with other prescription medications 

Barbiturates (Phenobarbital and Primidone)

Phenobarbital (Luminal, generic), also called phenobaritone, is a barbiturate anticonvulsant. Primidone (Mysoline, generic) is converted in the body to phenobarbital, and has the same benefits and adverse effects.

Barbiturates may be used to prevent grand mal (tonic-clonic) seizures or partial seizures. They are no longer typically used as first-line drugs, although they may be the initial drug prescribed for newborns and young children.

Side Effects. Phenobarbital has fewer toxic effects on other parts of the body than most anti-epileptic drugs, and drug dependence is rare, given the low doses used for treating epilepsy. Nevertheless, many patients experience difficulty with side effects.

Patients sometimes describe their state as "zombie-like." The most common and troublesome side effects are:

  • Drowsiness
  • Memory problems
  • Problems with tasks requiring sustained performance
  • Problems with motor skills
  • Hyperactivity in some patients, particularly in children and the elderly
  • Depression in some adults

When taken during pregnancy, phenobarbital, like phenytoin and valproate, may lead to impaired cognitive function in the child. There is some evidence that phenobarbitol may cause heart problems in the fetus.

Ethosuximide and Similar Drugs

Ethosuximide (Zarontin, generic) is used for petit mal (absence) seizures in children and adults when the patient has experienced no other type of seizures. Methsuximide (Celontin), a drug similar to ethosuximide, may be suitable as an add-on treatment for intractable epilepsy in children.

Side Effects. This drug can cause stomach problems, dizziness, loss of coordination, and lethargy. In rare cases, it may cause severe and even fatal blood abnormalities. Periodic blood counts are recommended for patients taking this drug.

Clonazepam

Clonazepam (Klonopin, generic) is recommended for myoclonic and atonic seizures that cannot be controlled by other drugs and for Lennox-Gastaut epilepsy syndrome. Although clonazepam can prevent generalized or partial seizures, patients generally develop tolerance to the drug, which causes seizures to recur.

Side Effects. People who have had liver disease or acute angle glaucoma should not take clonazepam, and people with lung problems should use the drug with caution. Clonazepam can be addictive, and abrupt withdrawal may trigger status epilepticus. Side effects include drowsiness, imbalance and staggering, irritability, aggression, hyperactivity in children, weight gain, eye muscle problems, slurred speech, tremors, skin problems, and stomach problems.

Lamotrigine

Lamotrigine (Lamictal, generic) is approved as add-on (adjunctive) therapy for partial seizures, and generalized seizures associated with Lennox-Gastaut syndrome, in children aged 2 years and older and in adults. Lamotrigine is also approved as add-on therapy for treatment of primary generalized tonic-clonic (PGTC) seizures, also known as “grand mal” seizures, in children aged 2 years and older and adults. Lamotrigine can be used as a single drug treatment (monotherapy) for adults with partial seizures. Birth control pills lower blood levels of lamotrigine.

Side Effects. Common side effects include dizziness, headache, blurred or double vision, lack of coordination, sleepiness, nausea, vomiting, insomnia, and rash. Although most cases of rash are mild, in rare cases the rash can become very severe. The risk of rash increases if the drug is started at too high a dose or if the patient is also taking valproate. (Serious rash is more common in young children who take the drug than it is in adults.) Rash is most likely to develop within the first 8 weeks of treatment. Be sure to immediately notify your doctor if you develop a rash, even if it is mild.

Lamotrigine may cause aseptic meningitis. Symptoms of meningitis may include headache, fever, stiff neck, nausea, vomiting, rash, and sensitivity to light. Patients who take lamotrigine should immediately contact their doctors if they experience any of these symptoms.

Gabapentin

Gabapentin (Neurontin, generic) is an add-on drug for controlling complex partial seizures and generalized partial seizures in both adults and children.

Side Effects. Side effects include sleepiness, headache, fatigue, and dizziness. Some weight gain may occur. Children may experience hyperactivity or aggressive behavior. Long-term adverse effects are still unknown.

Pregabalin

Pregabalin (Lyrica) is similar to gabapentin. It is approved as add-on therapy to treat partial-onset seizures in adults with epilepsy.

Side Effects. Dizziness, sleepiness, dry mouth, swelling in hands and feet, blurred vision, weight gain, and trouble concentrating may occur.

Topiramate

Topiramate (Topamax, generic) is similar to phenytoin and carbamazepine and is used to treat a wide variety of seizures in adults and children. It is approved as add-on therapy for patients 2 years and older with generalized tonic-clonic seizures, partial-onset seizures, or seizures associated with Lennox-Gastaut syndrome. It is also approved as single drug therapy.

Side Effects. Most side effects are mild to moderate and can be reduced or prevented by beginning at low doses and increasing dosage gradually. Common side effects may include numbness and tingling, fatigue, abnormalities of taste, difficulty concentrating, and weight loss. Serious side effects may include acute glaucoma and other eye problems. Tell your doctor right away if you have blurred vision or eye pain.  If used during pregnancy, topiramate may increase the risk for cleft lip or palate birth defects.

Oxcarbazepine

Oxcarbazepine (Trileptal, generic) is similar to phenytoin and carbamazepine but generally has fewer side effects. It is approved as single or add-on therapy for partial seizures in adults and for children ages 4 years and older.

Side Effects. Serious side effects, while rare, include Stevens-Johnson syndrome and toxic epidermal necrolysis. These skin reactions cause a severe rash that can be life threatening. Rash and fever may also be a sign of multi-organ hypersensitivity, another serious side effect associated with this drug. Oxcarbazepine can reduce sodium levels (hyponatremia). Your doctor may want to monitor the sodium (salt) level in your blood. This drug can reduce the effectiveness of birth control pills. Women who take oxcarbazepine may need to use a different type of contraceptive.

Zonisamide

Zonisamide (Zonegran, generic) is approved as add-on therapy for adults with partial seizures.

Side Effects. Zonisamide increases the risk for kidney stones. It may reduce sweating and cause a sudden rise in body temperature, especially in hot weather. Other side effects tend to decrease over time and may include dizziness, forgetfulness, headache, weight loss, and nausea.

Levetiracetam

Levetiracetam (Keppra, generic) is approved both in oral and intravenous forms as add-on therapy for treating many types of seizures in both children and adults.

Side Effects. These tend to occur mostly in the first month. They include sleepiness, dizziness, and fatigue. More serious side effects may include muscle weakness and coordination difficulties, behavioral changes, and increased risk of infections.

Tiagabine

Tiagabine (Gabitril) has properties similar to phenytoin and carbamazepine.

Side Effects. Tiagabine may cause significant side effects including dizziness, fatigue, agitation, and tremor. The FDA has warned that tiagabine may cause seizures in patients without epilepsy. Tiagabine is only approved for use with other anti-epilepsy medicines to treat partial seizures in adults and children 12 years and older

Ezogabine

Ezogabine (Potiga), a potassium channel opener, was approved in 2011 for treatment of partial seizures in adults. Ezogabine is used as an add-on (adjunctive) medication. Its most serious side effect is urinary retention. Patients should be monitored for symptoms such as difficulty initiating urination, weak urine stream, or painful urination. Other side effects may include coordination problems, memory problems, fatigue, dizziness, and double vision. In 2013, the FDA warned that this drug may cause retina abnormalities, vision loss, and skin discoloration.

Perampanel

Perampanel (Fycompa) was approved in 2012 as add-on treatment for partial onset seizures in patients age 12 years and older. Perampanel is the first in a new class of AEDs for uncontrolled partial epilepsy. It targets the AMPA glutamate receptor, which is involved in seizure activity. Perampanel is taken as a once-daily tablet. Common side effects may include dizziness, drowsiness, and fatigue. Perampanel also has a boxed warning to alert about potential risks of serious mood changes and mental disturbances including irritability, aggression, anxiety, and violent thoughts or behaviors.

Less Commonly Used AEDs

Felbamate. Felbamate (Felbatol) is an effective antiseizure drug. However, due to reports of deaths from liver failure and from a serious blood condition known as aplastic anemia, felbamate is recommended only under certain circumstances. They include severe epilepsy, such as Lennox-Gastaut syndrome, or as monotherapy for partial seizures in adults when other drugs fail.

Vigabatrin. Vigabatrin (Sabril) has serious side effects, such as vision disturbances, and is generally prescribed only in specific cases. It is sometimes given in low doses for patients with Lennox-Gastaut syndrome. Vigabatrin is also prescribed as a low-dose oral solution to treat infantile spasms in children ages 1 month to 2 years.

Clobazam. Clobazam (Onfi) is a benzodiazepine drug prescribed as add-on treatment for children with Lennox-Gastaut syndrome. In 2013, the FDA warned that clobazam can cause rare but serious skin reactions such as Stevens-Johnson syndrome and toxic epidermal necrolysis. The risk for these reactions is highest during the first 8 weeks of treatment. While these types of serious skin reactions can occur with other AEDs, they are not usually associated with benzodiazepine drugs.

Surgery and Devices

Surgery or surgically implanted neurostimulation devices may be options for patients with difficult-to-treat epilepsy that cannot be controlled with medications. .

Surgery

Surgery for epilepsy is brain surgery. There are three main types of surgery:

  • Resection surgery (lobectomy/lesionectomy)
  • Corpus callostomy
  • Hemispherectomy

The goal of surgery is to eliminate or at least reduce seizure activity while not causing any functional deficits, such as deterioration of speech or cognitive abilities. Even if surgery is successful, patients may still need to take medications for 1 – 2 years following surgery, to help maintain seizure control.

A surgical team will conduct a thorough evaluation prior to any surgery to make sure a patient is a good candidate. Various advanced imaging brain scan and mapping techniques are used to provide information on the exact area or areas in the brain that are triggering seizure activity. It is also important to identify a patient’s suitability for surgery based on psychological and other medical factors. Patients with significant psychiatric disorders, or other significant health problems, may not be suitable candidates
.

Brain
The major areas of the brain have one or more specific functions.

Resection Surgery. Cortical resection surgery involves removing a small part of the brain. The two types of resection surgery are lobectomy and lesionectomy.

Lobectomy involves removing an entire lobe of the brain. The most common and successful surgical procedure for epilepsy is anterior temporal lobectomy, which is performed when seizures originate in the temporal lobe. (Surgery is not as successful in epilepsies that occur in the frontal lobe.) It involves removing the front portion of the temporal lobe and small portions from the hippocampus and often the amygdala.

The hippocampus is contained in the temporal lobe and is a part of the brain that is involved in memory processing. It is part of the limbic system, which controls emotions. Temporal lobectomy successfully reduces or eliminates seizures in most patients, but can cause problems with memory and learning.



Click the icon to see an image of the limbic system.

Lesionectomies are performed to remove damaged or abnormal tissue that is causing seizure activity. These lesions may be congenital (present at birth) such as cortical dysplasia. Lesions may also be due to other acquired conditions such as strokes or head trauma.

Corpus Callostomy. Corpus callostomy disconnects the nerve connections between the right and left sides (hemispheres of the brain). The surgeon cuts into the band of tissue that connects these hemispheres to prevent seizures from becoming generalized and spreading from one side of the brain to the other. However, this procedure does not stop seizures from continuing to occur in one side of the brain.

Hemispherectomy. Children who have extremely severe seizures that originate on only one side of the brain may be candidates for hemispherectomy. This is a radical surgical procedure that involves removing a large portion of one side of the brain.

Neurostimulation Devices

Electrical stimulation of areas in the brain that affect epilepsy can help many patients with refractory epilepsy. Neurostimulators are sometimes called “pacemakers for the brain.” In 1997, the FDA first approved vagus nerve stimulation (VNS) for treating patients with partial epilepsy whose seizures were not helped by medication. In 2013, the FDA approved a second neurostimulation device, the RNS stimulator. Researchers are continuing to study other types of brain stimulation devices as treatments for epilepsy.

Vagus Nerve Stimulation (VNS). Electrical stimulation of the vagus nerve is an accepted therapy for severe epilepsy that does not respond to medication. The two vagus nerves are the longest nerves in the body. They run from the brain stem and down through each side of the neck, and then pass through the esophagus to the gastrointestinal tract. They affect swallowing, speech, and many other functions. They also appear to connect to parts of the brain that are involved with seizures. The VNS procedure is as follows:



Click the icon to see an image of vagus nerve stimulation.
  • A battery-powered device similar to a pacemaker is implanted under the skin in the upper left of the chest.
  • A lead is then attached to the left vagus nerve in the lower part of the neck.
  • The neurologist programs the device to deliver mild electrical stimulation to the vagus nerve. (Patients may also pass a magnet over the device to give it an extra dose if they sense a seizure coming on.)
  • The batteries wear out after 5 - 10 years and need to be removed and replaced by a simple surgical procedure.

VNS is FDA-approved for people with partial epilepsy and patients whose seizures are not helped by medication. According to the most recent guidelines from the American Academy of Neurology, there is weak evidence that VNS may help as added therapy for children with Lennox-Gastaut syndrome, and children and adults with generalized or partial epilepsy. There is also weak evidence that VNS may possibly help improve depression and mood problems in people with epilepsy. .

Vagus nerve stimulation can help reduce seizure frequency and how long seizures last. For side effects, VNS can cause shortness of breath, hoarseness, sore throat, coughing, ear and throat pain, or nausea and vomiting. These side effects can be reduced or eliminated by reducing the intensity of stimulation.

Some studies suggest that the treatment causes adverse changes in breathing during sleep and may cause lung function deterioration in people with existing lung disease. People who have obstructive sleep apnea should be cautious about this procedure. Turning off the VNS (for example before an MRI or surgery) may increase the risk for status epilepticus. (However, VNS may also be helpful for treating status epilepticus in some patients.)

Responsive Neurostimulation (RNS Stimulator). In 2013, the FDA approved a new type of neurostimulator device called the RNS stimulator. What makes RNS different from VNS is that it is a responsive stimulation system. RNS detects abnormal electrical activity in the brain and responds by sending electrical stimulation. In other words, the device can help stop seizures before they occur.

The RNS system is approved for adults with partial onset seizures that could not be controlled with two or more anti-epileptic drugs. The RNS Stimulator will not completely eliminate seizures, but can help reduce seizure frequency. In clinical trials, the most common side effect was infection at the implant site. Patients who have an RNS stimulator implanted cannot undergo MRI procedures.

Lifestyle Changes

Epilepsy is a chronic and usually lifelong condition. Seizures cannot be prevented by lifestyle changes alone, but people can make behavioral changes that improve their lives and give them a sense of control.

Avoiding Epileptic Triggers

In most cases, there is no known cause for epileptic seizures, but specific events or conditions may trigger them and should be avoided.

Poor Sleep. Inadequate or fragmented sleep can set off seizures in many people. Using sleep hygiene or other methods to improve sleep may be helpful.

Food Allergies. Specific foods are not generally associated with seizures but may have an effect in certain people. If you suspect that a food is a seizure trigger, try keeping a diary of what you eat and when your seizures occurred.  Also check with your doctor if certain foods may interact with any medications you are taking.

Alcohol and Smoking. Alcohol and smoking should be avoided. Light alcohol consumption does not usually increase seizure activity in people who are not alcohol dependent or sensitive to alcohol. Heavy alcohol use can trigger seizures, as can cigarette smoking.

Flashing Lights. Patients should avoid exposure to flashing or strobe lights. Video games have been known to trigger seizures in people with existing epilepsy, but apparently only if they are already sensitive to flashing lights. Seizures have been reported among people who watch cartoons with rapidly fluctuating colors and quick flashes.

Relaxation Techniques

Relaxation methods include deep breathing, biofeedback, and meditation techniques. No strong evidence supports their value on reducing seizures (although some people benefit), but they may be helpful in reducing anxiety.

Exercise

Exercise is important for many aspects of epilepsy, although it can be problematic. Weight-bearing exercise helps maintain bone density, which can be reduced by some medications. Exercise can help to prevent weight gain, which is a problem with some drugs. Exercise is also helpful for preventing depression and maintaining good emotional and psychological health.

People with epilepsy do need to take certain precautions to exercise safely. These include making sure not to get too hot or tired, which may trigger seizures. They should refrain from sports that might cause injuries to the head. Swimming is great exercise, but drowning is a serious risk for people with epilepsy. Make sure that you have a companion with you when you go swimming who can recognize the signs of a seizure and knows what to do if you have one. It’s also safest to swim at a pool that has a life guard on duty.

Dietary Measures

All patients should maintain a healthy diet, including plenty of whole grains, fresh vegetables, and fruits. In addition, dairy foods may be important to maintain calcium levels.

Fasting has been used to prevent seizures since ancient times. In the 1920s, a high-fat, no-sugar, low-protein diet, known as a ketogenic diet, was used to prevent seizures. Researchers are investigating whether a modified version of the Atkins diet (high protein, low carbohydrate) may help people with epilepsy.

Both the ketogenic diet and the Atkins diet can interfere with some anti-epileptic medications, such as topiramate. Talk to your doctor before beginning any special diet or a weight loss program.

The Ketogenic Diet

The ketogenic diet -- which is very high in fat (90%), very low in carbohydrates, and low in protein -- has been studied and debated for decades. It has proven to be helpful for many children with severe epilepsy that does not respond to AEDs. It is not clear why it works. The standard theory is that burning fat instead of carbohydrates causes an increase in ketones (chemical substances in the body that result from the breakdown of fat in the body). When excessive levels of ketones are produced, a metabolic state called ketosis happens. Ketosis appears to alter certain amino acids in the brain and to increase levels of the neurotransmitter gamma aminobutyric acid (GABA), which helps prevent nerve cells from over-firing.

Benefits. Studies report that about 10 - 15% of children who use the diet are seizure free after 1 year, while 30% are nearly seizure free. Some parents report that the diet helps improve their children’s alertness, even if seizures continue. Some children who try the ketogenic diet are able to stop or at least reduce their medications.

Typical Ketogenic Diet. (This diet must be professionally monitored! Parents can endanger their children if they try the program on their own without consulting a doctor or trained health expert.) The child fasts for the first 1 - 2 days, and then the diet is gradually introduced. The regimen uses small amounts of carbohydrates and large amounts of fats (up to 90%), with very few proteins and no sugar. Children generally consume 75% of their usual daily calorie requirements.

A typical dinner may include a chicken cutlet or piece of fish, broccoli with cheese, lettuce with mayonnaise, and a whipped cream sundae. Vegetables may include celery, cucumbers, or asparagus, cauliflower, and spinach. Breakfast might consist of an omelet, bacon, and cocoa with cream. (Artificial sweeteners are used for any desserts.)

The diet is very complex and difficult, as a slight deviation from the diet can provoke a seizure. Children cannot take medications that contain sugar (which is common in many drugs produced for children). Some sunscreens and lotions contain sorbitol, a carbohydrate that can be absorbed through skin. About half of patients find the diet too difficult or ineffective and stop it after 6 months.

Researchers are investigating a modified version of the popular Atkins diet, which does not require the caloric, protein, or fluid restrictions of the ketogenic diet. Unlike the traditional Atkins diet, the modified Atkins diet uses less carbohydrates and more fat intake. Early results indicate that it might be helpful for some patients with epilepsy. Another alternative is a low glycemic index diet, which contains even fewer carbohydrates than the Atkins diet. Still, parents should not put their children on these diets without support from a doctor.

Side Effects and Complications. To prevent serious side effects, children need regular monitoring by a doctor, especially when the ketogenic diet is first initiated.

Side effects or complications that may occur at the start of the diet include:

  • Acidosis, a build-up of acid in the blood and body
  • Low blood sugar (hypoglycemia)
  • Stomach upset
  • Dehydration
  • Lethargy

Side effects that may occur later on include:

  • Unhealthy cholesterol and lipid levels
  • Vitamin and mineral deficiencies (patients need to take supplements)
  • Kidney stones, which may be a complication of acidosis.
  • Slowing of growth (tends to occur more in younger children than older children)
  • Decreased bone density

Because most patients remain on the diet for only 2 years, the risks for potential long-term damage appear minimal.

Emotional and Psychologic Support

Many patients with epilepsy and parents whose children have epilepsy can benefit from support associations. These services are usually free and available in most cities.

Tips for Helping Children. Some of the following tips may help the child with epilepsy:

  • Children should be treated as normally as possible by parents and siblings.
  • Children should be assured that they will not die from epilepsy.
  • Most children will not have seizures triggered by sports or by any other ordinary activities that are enjoyable and healthy.
  • As soon as they are old enough, children should be active participants in maintaining their drug regimens, which should be presented in as positive a light as possible.

Therapies for Children and Adults. Many people with epilepsy contend with depression and other mental health issues. Psychological therapy may be helpful. Cognitive behavioral therapy offers a structured counseling program that helps people change behaviors associated with seizure triggers, such as anxiety and insomnia.

Resources

References

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Christensen J, Grønborg TK, Sørensen MJ, et al. Prenatal valproate exposure and risk of autism spectrum disorders and childhood autism. JAMA. 2013;309(16):1696-1703.

Christensen J, Vestergaard M, Mortensen PB, Sidenius P, Agerbo E. Epilepsy and risk of suicide: a population-based case-control study. Lancet Neurol. 2007;6(8):693-698.

Fiest KM, Dykeman J, Patten SB, et al. Depression in epilepsy: a systematic review and meta-analysis. Neurology. 2013;80(6):590-599.

Freeman JM, Kossoff EH, Hartman AL. The ketogenic diet: one decade later. Pediatrics. 2007 Mar;119(3):535-543.

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Harden CL, Hopp J, Ting TY, et al. Practice parameter update: management issues for women with epilepsy--focus on pregnancy (an evidence-based review): obstetrical complications and change in seizure frequency: report of the Quality Standards Subcommittee and Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology and American Epilepsy Society. Neurology. 2009 Jul 14;73(2):126-132.

Harden CL, Meador KJ, Pennell PB, et al. Practice parameter update: management issues for women with epilepsy -- focus on pregnancy (an evidence-based review): teratogenesis and perinatal outcomes: report of the Quality Standards Subcommittee and Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology and American Epilepsy Society. Neurology. 2009;73(2):133-141.

Harden CL, Pennell PB, Koppel BS, et al. Practice parameter update: management issues for women with epilepsy--focus on pregnancy (an evidence-based review): vitamin K, folic acid, blood levels, and breastfeeding: report of the Quality Standards Subcommittee and Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology and American Epilepsy Society. Neurology. 2009;73(2):142-149.

Hernández-Díaz S, Smith CR, Shen A, et al. Comparative safety of antiepileptic drugs during pregnancy. Neurology. 2012;78(21):1692-1699.

Hirsch LJ, Donner EJ, So EL, et al. Abbreviated report of the NIH/NINDS workshop on sudden unexpected death in epilepsy. Neurology. 2011;76(22):1932-1938.

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Review Date: 3/7/2013
Reviewed By: Harvey Simon, MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team. Author: Julia Mongo, MS. A.D.A.M. Editorial Update: 04/18/2014.
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