Epilepsy

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Epilepsy

WHAT IS EPILEPSY?

Epilepsy is characterized by unprovoked, recurring seizures that disrupt the nervous system and can cause mental and physical dysfunction. It is not a single disorder but rather a wide spectrum of problems. What all types of epilepsy share is an uncontrolled electrical discharge from nerve cells in the cerebral cortex, the part of the brain that integrates higher mental functions, general movement, the functions of the internal organs in the abdominal cavity, perception, and behavioral reactions.

Epilepsy is often classified in three ways in order to determine the right treatment:

Type of seizures. [ See Box Types of Epileptic Seizures.]
Specific syndromes that are associated with one or more of these seizure types. [See Box Epileptic Syndromes.]
Specific causes of the seizures, if known. [See What Are the Causes of Epilepsy?]

In the US more than 2.3 million people are affected by seizures, and an estimated 3% of the population (about 7.2 million people) will experience at least one seizure during their lifetime, not counting the 5% of children who have seizures caused by fevers. Epilepsy is decreasing in childhood but increasing in the elderly, probably because of mild strokes and cardiac arrest.

TYPES OF EPILEPTIC SEIZURES

Partial (Focal) Seizures

A partial, or focal, seizure is the more common type of epilepsy and is caused by a disorder of the neurons in a specific site on one side of the brain. It is further categorized as a simple partial, complex partial, or secondarily generalized seizure.

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 as deja vu, mild hallucinations, or extreme responses to smell and taste. After the seizure, the patient usually has temporary weakness in certain muscles.

Complex Partial Seizures. Slightly over half of the seizures in adults are complex partial types, and about 80% of these seizures originate in the temporal lobe, the part of the brain located close to the ear. Disturbances there can result in loss of judgment, involuntary or uncontrolled behavior, or even loss of consciousness. About 20% of these patients have seizures that start in the brain's frontal lobes.

Prior to the actual seizure, people sometimes experience a warning sign, known as an aura, which can be an odd odor, a feeling of warmth, or a visual or auditory hallucination. They then may lose consciousness briefly and appear to others as motionless with a vacant stare. Emotions can be exaggerated; some sufferers even appear to be drunk. After a few seconds, some may begin to perform repetitive movements, such as chewing or smacking of lips. Episodes usually last no more than two minutes, and people can have them infrequently or as often as every day. A throbbing headache may follow a complex partial seizure.

Secondarily Generalized Seizures. In some cases, simple or complex partial seizures evolve into what are known as secondarily generalized seizures. [ See Generalized Seizures below. ] The progress may be so rapid that the partial stage is not even noticed.

Generalized Seizures

Generalized seizures are caused by nerve cell disturbances that occur in more diffuse areas of the brain than do partial seizures. Therefore, they have a more serious effect on the patient. They are further subcategorized as tonic-clonic (or grand mal) or absence (petit mal) 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 rigidly for about 10 to 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 called stridor when the patient inhales. Spasms occur for about 30 seconds to a minute as the seizure enters the clonic phase , when 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 two to three 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. Petit mal or absence seizures are brief (three to 30 seconds) and may consist of only a short cessation of physical movement and loss of attention. They may even pass unnoticed by others. Small children may simply appear to be staring or walking distractedly. Petit mal may be confused with simple or complex partial seizures. In petit mal, however, a person loses consciousness and may experience attacks as often as 50 to 100 times a day. About 25% of patients with petit mal develop grand mal seizures.

Other Seizures

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 their tonic rigidity.

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

EPILEPSY SYNDROMES

Epilepsy is also classified by syndrome or grouped according to a set of common characteristics, such as the following:

Patient age
Type of seizure or seizures,
Whether a cause is known or not (idiopathic).

A few syndromes and inherited epilepsies are listed as follows. They by no means represent all epilepsies.

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 between four and eight months.

Benign Familial Neonatal Convulsions

Benign familial neonatal convulsions (BFNC) are a rare, inherited form of generalized seizures that occur in infancy.

Juvenile Myoclonic Epilepsy (Impulsive Petit Mal)

Juvenile myoclonic epilepsy, which is also called impulsive petit mal epilepsy, is characterized by generalized seizures, usually tonic-clonic signaled by myoclonia (jerky movements) or absences. This accounts for 7% of epilepsies and usually occurs in individuals between ages eight to 20.

Adult Myoclonic Epilepsy

Some research now suggests that adult myoclonic epilepsy may be a previously undescribed and distinct syndrome. It involves the development of generalized epilepsy of unknown causes in middle-aged adults.

Lennox-Gastaut Syndrome

Lennox-Gastaut syndrome is a severe form of epilepsy in young children that causes multiple seizures and some developmental retardation. 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 an inherited disorder occurring in children between the ages of six and 15. It usually involves tonic-clonic seizures and marked sensitivity to light flashes. Although the disease was previously considered to be progressive throughout life, current therapies have significantly improved its outlook.

Autosomal Dominant Nocturnal Frontal Lobe Epilepsy

Autosomal dominant nocturnal frontal lobe epilepsy is a rare, inherited syndrome that usually occurs during childhood, on average at 11 years (although onset varies widely within families). Seizures can be dystonic (twisting contractions) or tonic (muscle contractions), or involve thrashing around. They are brief, frequent, and occur in clusters during the night, but often subside with age.

Landau-Kleffner Syndrome

Landau-Kleffner syndrome is an epileptic condition that results in the inability to communicate either with speech or by writing ( aphasia).

WHAT ARE THE CAUSES OF EPILEPSY?

A seizure's cause can be determined for about 28% of partial epilepsy patients. In the rest, , however, epilepsy is idiopathic, which means that the cause is unknown. The age of seizure onset can sometimes offer a clue. In fact, idiopathic epilepsy is rare in children and young adults. According to a 2001 study, only 1% of cases in those age groups have no evident cause.

General Biologic Mechanisms Involved with Seizures

Epileptic seizures are triggered by abnormalities in the brain that cause a group of nerve cells in the cerebral cortex to become activated simultaneously, emitting sudden and excessive bursts of electrical energy that lead to seizures. Depending on the location in the brain where this electrical hyperactivity occurs, seizures have a wide range of effects on the sufferer, from brief moments of confusion to minor spasms to loss of consciousness.

Ion Channels. Sodium, potassium, and calcium act as ions in the brain. That is, 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 that can cause misfire and seizures. Abnormalities in the ion channels are believed to be responsible for many seizures.

Neurotransmitters. Abnormalities may occur in neurotransmitters, the chemicals that act as messengers between nerve cells. Two neurotransmitters are of particular interest:

The neurotransmitter gamma aminobutyric acid (GABA), which helps prevent nerve cells from over-firing. GABA deficiencies then, are of particularly interest to researchers in epilepsy.
Problems with the neurotransmitter serotonin are also being studied. This is an important brain chemical known as a neurotransmitter that is important for well being and associated behaviors (eating, relaxation, sleep).
Acetylcholine is a neurotransmitter in the cholinergic system, which is important for learning and memory, and is of interest in seizures.

Other Biologic Factors. Investigations of one novel mouse gene have suggested that unique biologic actions unrelated to ion channels may be responsible for some seizures triggered by sudden noises or visual stimuli. Research is needed to uncover these mechanisms.

Causes of Childhood Seizures

Febrile Seizures. Febrile seizures are caused by high fever and usually occur between the ages of three months and five years. Between 10% and 15% of children with epilepsy have a history of febrile seizures before they develop the disease. It should be strongly noted, however, that febrile seizures are quite common and occur in about 3% of all children under five years old. Nearly all are brief and have no long-lasting effect.

Genetic Factors. Epilepsy may be the most common genetic neurologic disease, but dozens of genetic syndromes representing a variety of seizure patterns may account for the different forms this disease takes. To date, however, researchers have identified only two epilepsy syndromes that are known to be caused by single genetic defects:

Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE). ADNFLE is now believed to be caused by an alteration in a receptor in the brain called neuronal nicotinic acetylcholine.
Benign familial neonatal convulsions (BFNC). BFNC appears to be caused by genetic defects that affect ion channels in nerve cells that carry potassium.

Vaccinations. In young children, high fever from a vaccination can, in rare instances, trigger seizures, which are almost always temporary and have no serious consequences. Some controversy arose a few years ago over the possibility that the DTP (diphtheria-tetanus-pertussis) vaccine might trigger epilepsy or other neurologic diseases. Some experts suggest that children who have neurologic events following their DTP shot already have a preexisting impairment such as epilepsy, which is revealed but not caused by the vaccine. Children with existing epilepsy may be at risk for seizures two or three days after the vaccination. Such a temporary worsening of their disease does not appear to pose a danger to the child. Infants with suspected neurologic problems may have their vaccinations delayed until their neurologic situation is clarified, but not beyond their first birthday.

Head Injuries in Infants and Children. Infants are at high risk for head trauma. In fact, one study suggested that any infant with scalp fracture that occurs with a hematoma may be at risk for brain injury. A hematoma occurs after an injury when blood collects in a mass that usually looks like a large purplish area. It should be noted that hematoma is quite common after delivery when it typically causes no problems.

Childhood Viral Infections. According to a 2001 study of 22 children with status epilepticus (sustained periods of convulsions), viral testing uncovered the presence of several pediatric viruses. Human herpesvirus 6 was particularly associated with severe seizures. Herpesvirus 6 is common in children and causes roseola infantum, an acute illness that can lead to high fever and skin rash but is usually benign.

Hydrocephalus and Shunts. Hydrocephalus is a condition that may occur in newborns and infants in which cerebrospinal fluid (CSF) accumulates in the brain, leading to excessive swelling of the spaces in the brain ( ventricles). The resulting pressure can damage the brain's tissue. Hydrocephalus itself is not commonly known to cause seizures, but its treatment, which involves insertion of a shunt, may be. The shunt is a device that drains the excess fluid from the brain to other parts of the body, as well as to a special reservoir that allows the shunt to be reached through the skin. One 2001 study noted that between 20% and 50% of shunted children may experience epileptic seizures, particularly if the shunt is placed before age two. More research on its relationship to epileptic seizures is clearly needed.

Cortical Dysplasias. This is an abnormality in fetal development in which the normal migration of nerve cells is altered.

Other Causes in Children. Seizures in infants and children may be due to birth defects, difficulties during delivery, or poisoning. Of note, melatonin, an herbal remedy available over the counter for sleep disorders, has been found to cause seizures in children who have existing neurologic problems.

Causes of Seizures that Are Specifically Adult-Onset

Alcohol Abuse. Alcohol abuse is one of the most common causes of adolescent- and adult-onset seizures. Seizures, nearly always generalized tonic-clonic, occur in about 10% of adults during withdrawal, and in about 60% of these patients, the seizures are multiple. The first seizure occurs between seven and 48 hours after the last drink, and the time between the first and last seizure is usually six hours or less. [For more information, see Report #56, Alcoholism.]

Head Injuries in Adults. Head injuries to adults can cause seizures, with the risk highest in severe head trauma. A first seizure related to the injury can occur years later. People with mild head injuries, which involve loss of consciousness for less than 30 minutes, have only a slight risk that lasts up to five years after the injury.

Sleep Disorders. Some sleep disorders, such as obstructive sleep apnea or narcolepsy, have been associated with seizures, although a causal relationship is unclear. In fact, sleep apnea and the hereditary nocturnal frontal lobe epilepsy have very similar symptoms (feeling of choking, abnormal motor activity during sleep, and excessive sleepiness during the day). In one 2000 study, one-third of patients with epilepsy that did not respond to medications were later diagnosed with obstructive sleep apnea. Some studies have found that when sleep apnea is treated in patients with both epilepsy and the sleep disorder, seizure activity decreases. More research is warranted on this subject.

Stroke. Seizure is also a symptom of a major stroke. In some cases, injury to the brain from small strokes may cause seizures. Studies report that between 15% and 23% of stroke patients consequently have seizures.

Other Causes in Adults. Other known or possible causes of epilepsy in teenage or adult years include the following:

Drug abuse or withdrawal from drugs.
Sudden withdrawal from certain antianxiety or antidepressant drugs.
Occupational exposure to environmental triggers. High exposure to certain chemicals has been linked with seizures. A 2000 study of utility company employees in Denmark revealed an association between high exposure to electromagnetic fields (EMF) and an increased risk of epilepsy and neurologic diseases that affected motor control.
Alzheimer's or other degenerative brain diseases in the elderly may cause seizures.
In developing nations, nervous system infection by tapeworm larvae is an important cause of epilepsy.

Causes of Seizures that Can Occur at Any Age

Infections of the Brain and Central Nervous System. Brain infections can cause seizures during the acute infection, and some cases are complicated by brain damage that can lead to recurrent seizures afterward. The most common central nervous system infections are encephalitis and meningitis. Encephalitis is a brain inflammation often caused by infections transmitted by mosquitoes. Meningitis is an inflammation or infection of membranes covering the brain or spinal cord. One study found an association between epileptic seizures and herpes simplex virus infections that occur in the central nervous system. More research is needed before any causative role can be proved.

Hypoglycemia. Seizures can be caused by hypoglycemia, a complication of diabetes in both children and adults.

Brain Tumors. Both cancerous and noncancerous brain tumors can cause seizures in children and adults.

Cavernous Angiomas. Cavernous angiomas are blood vessels that grow abnormally and, like a tumor, can put pressure on nerve tissue.

Pseudoepilepsy. Between 20% and 45% of cases of untreatable seizures have a psychologic rather than physical origin. In this form of epilepsy, known as pseudoepilepsy or psychogenic epilepsy, the patient has no conscious intent of forcing a seizure and does not show unusual emotional behavior or signs of hysteria. Pseudoepilepsy can usually be distinguished from true epilepsy using an electroencephalogram (EEG), which measures brain waves. The cause of pseudoepilepsy is unknown.

HOW SERIOUS IS EPILEPSY?

Emergency Situations

Acute Repetitive Seizures. Some patients occasionally experience seizures called acute repetitive, serial, or cluster seizures; these are two or more seizures occurring over minutes to hours separated by periods of consciousness. Left untreated, they can develop into status epilepticus, a very serious condition.

Status Epilepticus. Status epilepticus is often defined as recurrent convulsions that last for more than 20 minutes and are interrupted by only brief periods of partial relief. Some experts believe these criteria are too strict and that the condition should be diagnosed if seizures last at five minutes or more, or when the patient does not fully recover consciousness between two or more seizures. Although any type of seizure can be sustained or recurrent, the most serious form of status epilepticus is the generalized convulsive or tonic-clonic type. In more than a third of cases, status epilepticus occurs with the first seizure.

The trigger is often unknown, but can include the following:

Failure to take antiepileptic medications (comprising about a third of these events).
Abrupt withdrawal of certain antiepileptic drugs, particularly barbiturates and benzodiazepines.
High fever.
Poisoning.
Electrolyte imbalances (imbalance in calcium, sodium, and potassium).
Cardiac arrest.
Stroke. In one study, about 9% of stroke patients with seizures had status epilepticus, which was associated with higher disability after the stroke, particularly if these severe seizures occurred within a week of the stroke.
Low blood sugar in people with diabetes.

Status epilepticus is a serious condition that can lead to chronic epilepsy and can be life threatening. It occurs in 100,000 to 150,000 people in the US each year, over half of whom are children. Permanent brain damage or death can result if the seizure is not treated effectively; the longer the seizure lasts, the greater the danger. Mortality rates from this condition are about 10%. It should be noted that the high mortality rate is most likely due to a high incidence of myoclonic SEs in elderly adults after cardiac arrest. One study reported much lower mortality rates from SE when this condition is excluded.

Overall Outlook for Patients with Epilepsy

There are effective medical treatments available for epilepsy, and the majority of patients can control their seizures with a single drug and stop drug treatment completely after two seizureless years. Epileptic patients who are cured have a normal lifespan. This long-term survival rate is lower than average, however, if medications or surgery fail to stop the seizures. The lower survival rate is partly due to a higher-than-average risk for death due to accidents and suicide. If epilepsy is not effectively treated and the patient has continuing seizures, changes in the neurons may eventually cause intractable (also known as refractive) epilepsy, that is, epilepsy that is hard to control.

General Outlook for Childhood Epilepsy

Chance for Recurrence after a First Seizure. According to one 2000 study, about 64% children with one nonfebrile seizure have another one, and 74% who have more than one seizure are likely to have more seizures. Studies suggest that it is how frequent early seizures occur, not their total number or type, that determines whether a child will develop intractable epilepsy. For example, in one study, those at high risk were children whose first two seizures occurred within six months of each other.

Children with the following conditions are less likely to develop intractable epilepsy:

A first seizure that occurred after age two.
Normal electroencephalogram (EEG) readings that continue after treatment.
Normal intelligence.
A single generalized seizure, particularly petit mal.

Long-Term General Effects. In general, the long-term effects of seizures vary widely depending on the seizure's cause:

Febrile (Fever-Related) Seizures. Children with febrile seizures rarely have any long-term effects. In very rare cases, children experience severe fever-related seizures known as complex febrile convulsions. In such cases, there is a risk for brain injury that may lead to temporal lobe epilepsy, but this is very small. Such seizures last over 15 minutes, occur more than once within 24 hours, and may affect only one side of the body.
Idiopathic Epilepsy (Seizures of Unknown Causes). The long-term outlook for children with epilepsy of unknown causes is very favorable. One study reported that between 68% and 92% of such individuals were seizure-free after 20 years. In addition, a 2000 study reported that they had a survival rate no different from children without these seizures.
Epilepsy Caused by a Pre-Existing Condition. Children with epilepsy that is a symptoms of a another condition (for example, a head injury or neurologic condition) have higher mortality rates than the normal population, but their lower survival rates are due to the underlying condition not the epilepsy itself.

Withdrawal from Medication. Eventually, many children with epilepsy can go off medication. Children who tend to relapse after withdrawal from treatment are those with the following conditions or situations:

A family history of epilepsy.
Require multiple medications to control seizures.
Abnormal EEG readings after treatment has started.
Partial seizures.

Effect on Memory and Learning. The studies on the effects of seizures on memory and learning vary widely and depend on many factors. Some results include the following:

Interestingly, a 2001 study reported greater mental flexibility and memory capacity in some children with a history of febrile seizures compared to peers who did not have these seizures. Children with a history of febrile seizures before age one, however, were at higher risk for some learning deficits.
A number of studies have demonstrated no diminished intelligence in patients with epilepsy that occurs in the left temporal lobe (the left side of the brain where most complex partial types occur). A 2000 study suggested, however, that it may affect long-term memory. Patients with seizures originating on the left side of the brain may also have less well-developed language skills than those with right-side epilepsy.
A 2001 study suggested that children with generalized epilepsy who have petit mal (absence) seizures tend to retain their language skills and verbal memory, but some may have poorer testing scores, problems with nonverbal memory, and a slower recall of events.

In general, the earlier a child has seizures and the more extensive the area of the brain affected, the poorer the outcome. And, children with seizures that are not well-controlled are at higher risk for intellectual decline.

Social and Behavioral Consequences. Studies have noted that children with epilepsy perform worse on behavioral tests than do other children. In a well-conducted 2000 study, girls with severe epilepsy had the highest rate of behavioral problems (and they worsened over time) compared to boys and girls with mild or moderate epilepsy and all children with asthma, another chronic illness. In another study, although there were no differences in intelligence, adults with previous epilepsy (even if they no longer had seizures) were less likely to attain higher-education degrees and were slightly more likely to be unemployed, unmarried, and childless compared to the general population.

General Outlook for Adult Epilepsy

Chance for Recurrence. Adults whose first episode occurs when they are over 59 years of age have a higher risk for recurring seizures than do younger adults. Some studies have indicated that recurrence is least likely in adults with the following combination of factors:

A primary generalized seizure.
A seizure-free period of between two and five years.
A normal EEG reading.
Only a single-drug treatment.

In one study of adult-onset epilepsy, it was discovered that after one year of treatment, 70% of patients experienced complete control of their seizures, 14% had occasional seizures, and 16% were unable to control the seizures.

Effect on Mental Functioning in Adults. The effects of adult epilepsy on mental functioning are not clear. In fact, in a 2001 study IQ scores increased in adults with recurrent seizures during the trial period. However, more research is needed in this area, as results have been contradictory. For instance, one 2000 study yielded the opposite result, reporting that intelligence scores start declining with long duration of adult epilepsy.

Overall Physical Effects. In a major 2000 survey, 46% of the respondents with epilepsy described their overall health as "fair" or "poor" compared to 18.5% of those who did not have epilepsy. People with epilepsy also report a higher frequency of pain, depression, anxiety, and sleep problems. In fact, their overall health state is comparable to people with other chronic diseases, including arthritis, heart problems, diabetes, and cancer.

Emotional Consequence. About 25% to 75% of adults with epilepsy show signs of depression. They also have a higher than average risk for suicide. The most common emotional responses are the following:

Fear of the unexpected seizure.
Acute humiliation after a seizure, particularly if incontinence occurs.
Feelings of alienation at work and in social situations.

Emotional difficulties increase if epilepsy becomes chronic. In one study, the intensity of the negative emotional response was directly related to the intensity and frequency of the attacks.

Effect on Sexual and Reproductive Health

Effects on Sexual Function. There have been studies suggesting that up to two-thirds of patients with epilepsy experience sexual disturbances, including impotence in men. There are various reasons for this:

Epilepsy in childhood may cause disturbances in hormones regulating puberty.
Persistent seizures in adults may be associated with other hormonal and neurologic changes that contribute to sexual dysfunction.
Negative emotions due to epilepsy can reduce sexual drive.
Medications may be responsible for many of these cases, although newer agents may reduce this problem.

Effects on Female Fertility. Studies have been conflicting on the effects of fertility from epilepsy. In one 1998 study of women with epilepsy, however, fertility rates were 33% lower than among women in the general population. Lower rates may be due to antiepileptic drugs or to social factors, such as marriage at an older age. One 2001 Italian study reported greater number of hormonal abnormalities among women with epilepsy, particularly polycystic ovary syndrome, which can contribute to the higher incidence of infertility reported. On the other hand, a 1998 study reported that fertility rates in women with epilepsy are equal to that in the general population except in women whose seizures had caused mental disabilities.

Effects on Pregnancy. In women who become pregnant, there is a risk for uncontrolled seizures and birth defects from antiseizure medications. In studies of women who were carefully monitored, however, 95% of pregnancies (which is close to normal) had favorable outcomes. [ See Box The Pregnant Woman with Epilepsy, below.]

Injuries and Accidents

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. (Epilepsy patients who take the drug phenytoin have an even higher risk, since the drug can cause osteoporosis.)

Accidents while Driving. Needless to say, seizures can be very dangerous if they occur while a person is driving. Being unable to drive is an extremely distressing and severe component of the disorder. In one study, over 25% of people with uncontrolled epilepsy who drove had had an accident at some time because of a seizure, and over half of these accidents had resulted in injuries to the patients or others. Unfortunately, in spite of these events, 30% of these patients had driven within the past year, and most had driven at least once a week.

Four factors help predict who may safely drive:

A long duration between seizures. In one 1999 study, being seizure-free for six months reduced the risk for accidents by 85%, and being seizure-free for a year lowered the risk by 93%. State laws restricting driving in people with seizures vary from requiring seizure-free periods of three months (which is too short for protection) to 18 months.
Having few seizure-related accidents.
Having a reliable pre-seizure warning sign, such as an aura.
Recently having reduced or changed medications.

Accidents while Swimming. Swimming poses another danger for people with epilepsy, particularly those with tonic seizures, which can cause the diaphragm to expel air quite suddenly. People with epilepsy who swim should do so under the following conditions:

Always with a knowledgeable, competent, and experienced companion or supervisor, one to one is best.
Avoid deep and cloudy water. A clear swimming pool is best.

HOW IS EPILEPSY DIAGNOSED?

Physical Examination and Medical History

A diagnosis of epilepsy is often made during an emergency visit for a seizure. If a person seeks medical help for a previous or suspected seizure, the physician will take a complete medical history, including a history of seizure events, from either the patient or the parent.

One interesting study suggested that a physician might be able to identify the location in the brain where the seizure is originating by watching the patient wipe his or her nose. A runny nose is common after a temporal lobe seizure but not after seizures in other locations. Furthermore, the hand with which the patient wipes the nose coincides with the side of the brain in which the seizure occurs.

Ruling Out Serious Causes

Health- or life-threatening causes of seizures should first be ruled out. They include the following:

Alcohol withdrawal.
Infections (encephalitis or meningitis).
Head injuries.
Poisoning.
Drug overdose.
Hypoglycemia.
Stroke. Major and minor stokes are important causes of seizures in older people. Small strokes, called transient ischemic attacks, are often difficult to distinguish from mild epileptic seizures, and, in fact, a first seizure in an adult might be a precursor to stroke.
Cardiac arrest.

Ruling Out Conditions with Similar Symptoms

Syncope. Syncope, a brief lapse of consciousness in which blood flow is reduced to the brain, can mimic epilepsy and is misdiagnosed as epilepsy in many cases. A misdiagnosis of epilepsy in such patients can cause serious problems. Research continues to suggest that taking the patient's history and giving a physical exam rather than administering an assortment of cardiac tests is the most effective way to diagnose syncope.

Migraines. Migraine headaches, particularly migraine with auras, may sometimes be confused with epilepsy. 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 toward one side.

Panic Attacks. One study reported on patients with partial seizures that resembled panic disorder. Symptoms of panic disorder 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, and derealization or depersonalization.

Narcolepsy. Narcolepsy, a sleep disorder that causes a sudden loss of muscle tone and excessive daytime sleepiness, can be confused with epilepsy.

Electroencephalogram

The most important diagnostic tool for epilepsy is an electroencephalogram (EEG), which measures brain waves. 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 physician will want a day-long recording. Portable EEG units can be used to monitor patients throughout normal activities. EEGs are not foolproof; in one study half of people who had experienced an epileptic seizure showed a normal EEG reading. For example, juvenile myoclonic epilepsy, a syndrome characterized by generalized seizures, may be misdiagnosed on EEG for years as partial seizures.

Imaging Techniques

Computerized Tomography (CT) Scans. The doctor will usually order brain scans using computerized tomography (CT) for most adults and children with a first seizure. 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.

Other Advanced Imaging Techniques. More advanced scanning techniques, particularly magnetic resonance spectroscopy (MRS), positron emission tomography (PET), and single-photon emission computer tomography (SPECT), are becoming important tools for epilepsy researchers. They are also useful for detecting abnormalities, such as changes in brain activity, damaged or scarred locations in the brain where partial seizures are triggered, or tumors and other abnormalities that may be causing seizures. These images may help to determine if the disorder is treatable by surgery and may be used as a guide for surgeons.

Polysomnography

Some researchers recommend polysomnography for certain patients. This test is used to detect sleep disorders, such as obstructive sleep apnea, that is associated with epilepsy in some people.

Investigative Diagnostic Approaches

Low brain levels of the neurotransmitter gamma aminobutyric acid (GABA) are associated with an increased risk of seizure recurrence. Some researchers suggest that measuring GABA levels, along with EEG recordings, may help in assessing the risk of recurring seizures and in identifying those patients who could benefit from drugs that stimulate GABA function.

WHAT IS THE IMMEDIATE TREATMENT FOR EPILEPTIC SEIZURES?

Managing the Onset of a Seizure Outside the Hospital

When a person experiences a seizure, caretakers or bystanders cannot stop it, but they can prevent serious injury.

First, it is extremely important for caregivers to remain calm and not panic. They should 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 an old wives' tale that people having seizures will swallow their tongues.
Turn the victim 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 seizure victim alone. Anyone nearby should call 911 and the patient should be taken to an emergency room under the following circumstances:

If any seizure lasts beyond two or three minutes.
If the patient has been injured.
If the patient is pregnant.
If the patient is diabetic.
If parents or bystanders are at all uncertain.

Children with seizures caused by fever rarely require any treatment other than taking precautions that will prevent obstruction and reduce the fever. And a 2001 study revealed that among adult patients with known epilepsy who refused to go to the hospital following a seizure, only 5.8% had a subsequent seizure during the study's three-day follow-up period. Hospitalization then may not be necessary in many patients whose seizure is not severe or repetitive and who have no risk factors for complications. All patients or caregivers, however, should contact their physician in the event of a seizure.

Drugs Used for Managing an Acute Repetitive Seizures

The initial treatment for acute repetitive seizures, two or more seizures that occur over minutes to hours separated by periods of consciousness, are antianxiety drugs known as benzodiazepines. They include diazepam (Valium, Diastate) or lorazepam (Ativan). These drugs are available in the following forms:

Tablets taken orally or under the tongue (sublingual). Oral tablets are difficult to give a patient who is convulsing, however.
Rectal solutions, gels, or suppositories. Rectal administration is preferred. Solutions and gels work faster than suppositories. Diastat is rectal gel form of diazepam, and though more expensive than rectal solutions, it can be administered at home by a trained caregiver. The gel is safe and effective in reducing seizure frequency, particularly in children, and it may help prevent status epilepticus. Studies suggest that it significantly reduces the rate of hospitalization, and may even prove to be an alternative to drug therapy among children with prolonged or repetitive seizures.

Treatment of Status Epilepticus

The treatment goals of status epilepticus are the following:

Stop the seizures.
Prevent recurrence.
Determine and prevent any factors that might have triggered it.
Manage any complications.

Initial Management. The earlier a patient is treated, the better the results. In one study, seizures stopped in 80% of patients who were treated within 30 minutes. Only 40% of patients responded when they were treated after two hours. Initial management of status epilepticus consists of the following:

Administer any seizure medications [ see below ].
Support systems to maintain or attain normal breathing, blood pressure, electrolyte balances, body temperature, and heart functions.
Oxygen for patients who may need it.
Attention by medical personnel trained to determine any treatable cause of status epilepticus, such as drug withdrawal, low blood sugar, infection, substance abuse (particularly cocaine), or eclampsia (elevated blood pressure induced by pregnancy).

Medications for Status Epilepticus. One or more of the following medications may be used initially:

Benzodiazepine. An intravenously administered or injected benzodiazepine such as lorazepam (Ativan), diazepam (Valium), clonazepam, or midazolam (Versed) is usually used. Lorazepam or clonazepam is now preferred since they have a longer duration of action. Of note, midazolam is the only benzodiazepine available as a muscular injection and may prove to be effective for children, as safe as other benzodiazepines and safer than barbiturates.
Phenytoin or Fosphenytoin. Many physicians use phenytoin or fosphenytoin if seizures are not controlled by a benzodiazepine. These drugs must be prescribed with caution for patients who have liver and blood abnormalities or certain heart arrhythmias. Fosphenytoin is similar but has a faster action and is safer than phenytoin.
Phenobarbital. Although effective, barbiturates, such as phenobarbital (Barbita, Luminal), can reduce consciousness, blood pressure, and respiratory rate and are generally used only when other drugs have failed.

Other medications or higher doses may be used for status epilepticus that does not respond to initial treatments. They include:

Higher-dose barbiturates.
Higher-dose intravenous benzodiazepines. In one study midazolam, the injected benzodiazepine, was as effective and possibly safer than propofol, an intervenous sedative also used for uncontrolled status epilepticus [ see below ].
Propofol (Diprivan), an intravenously administered sedative.

All of the medications mentioned carry a risk for hypotension, an abrupt and possibly dangerous drop in blood pressure, which may require treatment.

WHAT ARE THE GENERAL GUIDELINES FOR LONG-TERM TREATMENT OF EPILEPSY?

Drug Treatment Success Rates

In one 2000 study that followed over 500 patients for three to five years, 63% of patients treated with medications become seizure-free during that period. In the same study, drugs failed to control epilepsy in about 30% of patients. Those with the poorest chances of success were those who started drug treatment after more than 20 seizures and those who failed to exhibit any benefit from their initial drug regimen. (In the latter case, subsequent drugs worked in only 11% of patients.)

Indications for Drug Treatment

Drug treatment is usually initiated or strongly considered for the following patients:

Children and adults who have had two or three seizures, unless there is either a long separation between seizures or the seizure is provoked by an injury or other specific causes.
Anyone after a single seizure if tests reveal any brain injury or if specific syndromes put a person at special risk for recurrence, for instance, in cases of myoclonic epilepsy.
Some physicians believe that any adult who has a first seizure should begin ongoing treatment, since another event is likely in 30% to 70% of these patients. In one study, when young adults were given a single drug (usually carbamazepine) after a first generalized seizure, only 22% had a subsequent seizure compared to about 70% of those who were not given treatment.

Determining a Drug Regimen

Most epileptic seizures can be controlled using a single-drug regimen. The specific drugs and whether more than one should be used are determined by various factors, including the patient's age and the seizure's type, frequency, and cause. Patients generally begin with low doses and build up until the seizures are controlled or a toxic reaction occurs. A drug's failure to reduce seizures can be attributed to factors such as:

The wrong dose level.
Improper timing.
Introducing the medication too rapidly.
Not managing conditions that triggered the seizure.
Instability of the drugs. Many of the tablet forms disintegrate easily with moisture, so pills should be stored in a dry place, not in the bathroom, and kept away from heat.
Noncompliance. This is a serious problem, particularly in young people. It is extremely important to take medication as prescribed by the physician, since studies have shown that uncontrolled epileptic attacks lead to changes in the neurons that may cause intractable epilepsy. In young people, positive attitude, continued support from family and health care providers, emotional well-being, and good treatment results are among the factors that can increase patient compliance, while unhealthful behaviors, such as smoking and alcohol use, can have a negative effect.

The physician should first address these issues. If the patient still does not respond, the doctor will usually try a different drug. If this fails, one or even two additional drugs at a time may be used. It should be noted that, while many drugs are now available for epilepsy, if seizures do not respond to the first few, the odds of a fourth or fifth working diminish greatly, and the patient should ask about surgical alternatives [ see below ].

Monitoring Effects

During the first few months of therapy, the physician will probably monitor drug levels in the blood once or twice in order to adjust dosages, to check for drug complications, and to be sure the patient is complying with the regimen. Many experts feel, however, that these blood tests are a less reliable indicator of problems than the patient's own self-observations of his or her responses 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.

Side Effects

All antiepileptic drugs have side effects, which vary depending on the drug. Increasingly, however, drugs are being designed to specifically target mechanisms causing seizures and should have fewer widespread effects. The complexity and potential severity of side effects are amplified when more than one drug is used. Seizures themselves can be a side effect of antiseizure drugs. [For specific side effects, see What Are the Specific Medications Used for Epilepsy?, below.]

There are some common problems from many of the common medications given for epilepsy. They include the following:

Fatigue.
Decreased appetite.
Possible bone and mineral disorders. These can often be controlled with calcium or vitamin D supplements.
Severe allergic reactions (particularly from particularly phenytoin, carbamazepine, phenobarbital, and primidone). They can include severe skin rashes, fever, and occasionally even inflammation or swelling of the liver, kidneys, or lymph nodes. One study reported benefits from administration of intravenous immune globulin (IVIG).

Drug Interactions

Antiseizure medications interact with many other drugs, making them more or less potent, so it is very important that patients inform their physician of everything they are taking, including over-the-counter medications and vitamins. Some specific interactions are covered in discussions of individual agents and many of the drugs used for epilepsy have some common effects on other medications. Several antiepileptic drugs reduce the effectiveness of oral contraception, for example. Other drugs that are affected by many of the antiepileptic medications are erythromycin and some drugs used to treat asthma, ulcers, and heart disease. [For specific interactions, see What Are the Specific Medications Used for Epilepsy?, below.]

Discontinuing Drug Therapy

An estimated 60% of all patients treated effectively can stop medication from within five to 10 years. If a patient has not had seizures for two years, the physician will often consider withdrawal from drug therapy. Some physicians prefer to wait until as many as five seizure-free years have passed. Attempts to halt drugs should be done during periods when seizures will cause the least harm. For instance, the best time to test the effects of drug withdrawal in teenagers might be about a year before they are eligible to drive.

Indications for Surgery

Both the Centers for Disease Control and Prevention and the National Association of Epilepsy Centers recommend that patients who have not responded to medications after a year should be referred to an epilepsy center to determine the next course of treatment. Surgery is an excellent option for appropriate patients with epilepsy in the temporal lobe (where most complex partial seizures occur). In general, about 75% of appropriate patients can expect at least partial remission at experienced centers, with one center reporting success rates of 92%. (About 20% of patients, relapse, however.) Yet despite these high successes and the significant chance for failure after trying four or five drugs, physicians now wait an average of 15 to 19 years before they consider a surgical alternative. Younger people are preferred of older adults as candidates for surgery because older people have more difficulty with rehabilitation.

Treatment for Special Population Groups

Treatment of Specific Seizure Syndromes Infants and Small Children

Infantile Spasms. Infantile spasms are treated with vigabatrin, adrenocorticotropic hormone (ACTH), or valproate. Some experts recommend that vigabatrin be given first and ACTH administered 10 to 14 days later. In one small study, no infants who were given this combination relapsed after four months. Newer drugs, such as topiramate and zonisamide, may also be effective for this problem.
Prolonged Febrile Seizures. Prolonged febrile seizures in infants and small children may be treated with intravenous benzodiazepines, usually diazepam. One study found that nasally administering midazolam (a newer benzodiazepine) was effective for managing febrile seizures in children. It is absorbed quickly and is as safe as diazepam. With the proper instruction, it can also be administered by caregivers at home.

Treatment of the Elderly. Antiepileptic drugs interact with many other agents 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. Standard drugs are usually effective, while safe, newer ones (including gabapentin, lamotrigine, oxcarbazepine and gamma-vinyl-GABA) may sometimes prove to be useful as sole therapy. These newer drugs also increase patient compliance, in that they have fewer side effects than the older ones.

Treatment of Women. Hormonal fluctuations affect epilepsy in about a third to a half of female patients. Estrogen appears to increase activity and progesterone reduces it. The effect of pregnancy on women with epilepsy is complex. [ See Box The Pregnant Woman with Epilepsy, below.] The following treatments may help or affect women with epilepsy:

Hormonal Agents that Suppress Ovulation. When seizures in women are worsened by hormonal changes, such as during the menstrual cycle, suppressing ovulation may be recommended using drugs called gonadotropin-releasing hormone agonists.
Hysterectomy. Women with epilepsy who no longer wish to bear children may consider hysterectomy (surgical removal of the uterus) or oophorectomy (surgical removal of the ovaries). Each of these treatments must be accompanied by estrogen replacement therapy.
Oral contraceptives. Antiseizure medications affect many oral contraceptives (OCs). Carbamazepine, phenytoin, phenobarbital, primidone, oxcarbazepine, and topiramate reduce the effects of OCs. Valproate does not, and may even increase hormonal levels. Gabapentin, lamotrigine, tiagabine, and vigabatrin may also prove to be safe with OCs, but more research is needed. The best contraceptive agents for women with epilepsy at time may be progestins. Injected progestins may actually help prevent seizures in some cases.

THE PREGNANT WOMAN WITH EPILEPSY

Effects of Epilepsy on the Pregnant Patient and the Fetus

Effects of Seizures. Isolated seizures do not appear to pose any adverse effects to the mother and unborn child, but repeated seizures and status epilepticus can lead to great dangers. In one study, the effect of epilepsy on complications during pregnancy was the same as in non-epileptic women except for a higher rate of premature deliveries (8.2% in the women with epilepsy).

Effects of Medications. All standard antiseizure drugs pose a significant risk for birth defects, which include malformations of the face and hands or more serious effects on the heart or mental development. The more medications required, the higher the risk. (Epilepsy itself, however, does not appear to pose any higher risk for birth defects in the child.) Pregnant women who need to continue medication should be on the lowest possible dose of a single type of drug, if feasible. [ See Drugs Used During Pregnancy, below.]

Effect of Pregnancy on Seizure Frequency

The frequency and intensity of seizures vary widely in women with epilepsy. About 25% of pregnant women with epilepsy face an increase in events, and the risk is highest in those who have more than one seizure per month prior to becoming pregnant. In most cases, however, there is no change at all. Some pregnant women even have a decrease in seizures. The risk is lower in women who experience less than one seizure in the nine months prior to becoming pregnant. The following conditions may contribute to an increase in seizures during pregnancy:

Nausea and vomiting. (Vitamin B6 and antihistamines may help with nausea.)
Fluid retention.
Higher estrogen levels.
Psychological and emotional stress.
Medication noncompliance from fear of side effects.
Problems with sleeping.
Changes in absorption of anticonvulsants.

Steps Taken for Women who Want to Become Pregnant

A woman who wishes to become pregnant and has been seizure-free for two or more years may attempt to discontinue drugs.
If she has not been seizure-free, she should continue medications but try to reduce them to a single agent, if possible.

Steps Taken During Unplanned Pregnancy

If a woman taking antiseizure medications has an unplanned pregnancy, there is no point in switching medications right away, since the effects of the drugs last for 10 weeks.
She should, however, be carefully monitored for both drug levels and any abnormalities in the fetus. Ideally, drug levels should be measured every one or two months or more often if seizures are not controlled. Dosage levels should be adjusted accordingly.
She should also be carefully monitored with ultrasonic evaluation and amniocentesis (visual tests and examination of the fluid in the womb for birth defects and other fetal problems).

Drugs Used During Pregnancy

It is very difficult to determine which drugs are safest for pregnant women because most researchers (and patients) do not want to take chances testing unknown agents on unborn children. Some physicians believe carbamazepine is the safest agent for pregnant women at this time, although evidence on any drugs used during pregnancy is weak.

The risk for malformation is higher when more medications are used. For example, there is a 3% risk of birth defects with women who use one anticonvulsant; this risk increases to 20% when four drugs are used. Birth defects are more likely to occur when drugs are administered within the first trimester of pregnancy. The pregnant woman should use the most effective anticonvulsant medication for her type of epilepsy at the lowest dose possible to control seizures.

Birth Defects Associated with Medication. The most common birth defects related to anticonvulsant drugs are:

Cleft lip or palate (risk from phenobarbital, phenytoin, valproate).
Genital or urinary abnormalities (risk from most standard agents).
Neural tube defects (NTD) in the skull or spinal column (risk of 2% with valproate and 1% with carbamazepine). These complications are most often due to lower folic acid levels caused by both pregnancy itself and antiseizure drugs. Supplements can help prevent this problem. Folic acid is recommended for all pregnant women, in any case, and those with epilepsy should take a 5 mg supplement of folic acid at least three months before conception, if possible, as well as during the first trimester.
Mental impairment (known risk with phenytoin and valproate; inconclusive in carbamazepine and phenobarbital).
Heart defects (risk from phenobarbital, phenytoin, valproate).
Many of the major antiseizure drugs also cause a deficiency in vitamin K clotting factors that increases the risk for hemorrhage in the newborn, so treatment with vitamin K during the last month and a single dose given to the newborn is recommended.

Labor and Delivery

Seizures occur during labor and after delivery in a small percentage of women with epilepsy. The following labor complications are more common among pregnant women with epilepsy: vaginal bleeding, anemia, and preeclampsia (acutely elevated blood pressure in the third trimester). If seizures occur during labor, they are generally treated intravenously with benzodiazepines or phenytoin. If tonic-clonic seizures, absence seizures, or status epilepticus occur, a cesarean section may be appropriate.

Postnatal Care

Monitoring the Infant. The infant should be thoroughly examined for any malformations. Also, if the mother was given phenobarbital or primidone while pregnant, the infant should be monitored for up to eight months to see if withdrawal symptoms develop. Drug dosages will also need to be adjusted for the mother after delivery.

Breast Feeding. Women on antiseizure agents can usually nurse their babies, since usually only a small amount of the drug enters the child. The lowest levels are with phenytoin and valproate. (Women taking phenobarbital are usually advised not to nurse.) A mother should watch for signs of lethargy or extreme sleepiness in her infant, which could be caused by her medication.

WHAT ARE THE SPECIFIC MEDICATIONS USED FOR EPILEPSY?

Standard Medications

According to a 2000 survey of drug treatment options, the antiepilepsy drug arsenal has nearly doubled in size since 1993. As a result, physicians have been able to offer many of their patients drugs with improved effectiveness, tolerability, and safety. Depending on the seizure type, certain standard antiseizure medications are usually used first for epilepsy (called first-line agents). If they fail or if the patient becomes tolerant to the primary medications, then newer so-called add-on or second-line drugs are tried, usually in combination with standard drugs [ see table below ]. The lines are beginning to blur, however. In one 2000 study, patients who took newer drugs initially had as good a response as those who took the standard medications as their first agent.

Drugs Used According to Type of Seizure and Epileptic Syndrome
Type of Seizure and Epileptic Syndrome	First Line Drug (Generally, the first drug tried)	Second Line or Add-on Drug (Those tried when first-line drugs fail)
Primary Generalized Seizures
Absence (petit mal) seizures	Ethosuximide, valproic acid (divalproex sodium may be better tolerated). Note: Carbamazepine and phenytoin are contradicted.	Lamotrigine.
Myoclonic seizures	Valproic acid (or divalproex sodium) Note: Carbamazepine and phenytoin can actually aggravate these seizures.	Acetazolamide, clonazepam, lamotrigine, primidone, zonisamide, topiramate levetiracetam.
Tonic-clonic (grand mal) seizures	Valproic acid (or divalproex sodium), carbamazepine, phenytoin.	Lamotrigine, phenobarbital, primidone.
Absence epilepsy with onset in childhood	Ethosuximide. Note: Carbamazepine and phenytoin are contradicted.	Valproic acid (or divalproex sodium), lamotrigine.
Absence epilepsy with onset in adolescence	Valproic acid (or divalproex sodium). Note: Carbamazepine and phenytoin are contradicted.	Ethosuximide, lamotrigine.
Juvenile myoclonic epilepsy	Valproic acid (or divalproex sodium). Note: Carbamazepine and phenytoin are contradicted.	Acetazolamide, clonazepam, primidone, lamotrigine, topiramate.
Infantile spasms (West's syndrome)	Corticotropin, vigabatrin.	Clonazepam, valproic acid (or divalproex sodium), zonisamide.
Lennox-Gastaut syndrome	Valproic acid (or divalproex sodium), lamotrigine.	Carbamazepine. clonazepam, felbamate, phenobarbital, primidone, topiramate, or vigabatrin may be used alternatively.
Partial Seizures
Simple partial seizures, complex partial seizures, secondarily generalized tonic-clonic seizures, and partial epileptic syndromes	Carbamazepine, phenytoin, oxcarbazepine (in adults).	A 2001 comparison trial on add-on agents reported the following success rates: 12% to 20% for gabapentin, lamotrigine, zonisamide, and tiagabine. 27% to 29% for levetiracetam, topiramate, oxcarbazepine. Phenobarbital, primidone, valproic acid (or divalproex sodium), or methsuximide may also be used.
Original data from table Patients with Refractory Seizures, The New England Journal of Medicine, Vol. 340, No. 20, May 20, 1999. By permission of the author Orrin Devinsky, MD.

Carbamazepine

Carbamazepine (Tegretol, Carbatrol) is an effective anticonvulsant and specific analgesic when used alone or with other drugs. Carbamazepine also has the added benefit of relieving depression and improving alertness. Many physicians prefer carbamazepine for children; in fact, a chewable form is now available that will make it even easier to take.

This drug is used to prevent the following seizures or epilepsy syndromes:

Partial seizures, especially complex partial seizures. Patients with partial seizures tend to tolerate this drug better than others, but individual reactions vary.
Grand mal seizures.
Combinations of grand mal and partial seizures.
Autosomal dominant nocturnal frontal lobe epilepsy (an inherited disorder).

This drug is not useful for the following seizures:

Petit mal seizures.
Myoclonic seizures.
Atonic seizures.

Side Effects. Different side effects may develop or resolve at different points in the treatment duration.

Initial side effects may include:

Double vision, headache, sleepiness, dizziness, and stomach upset. These usually subside after a week and can be greatly reduced by starting with a small dose and building up gradually.
Some people experience visual disturbances, ringing in the ears, agitation, or odd movements when drug levels are at their peak. The extended-release form of carbamazepine (Carbatrol) may help reduce these symptoms.

Serious side effects are less common but can include the following:

Skin reactions develop that are so severe that the drug has to be discontinued in about 6% of patients.
Water retention can be a problem in older people.
Hormonal changes, particularly increased male hormones in both men and women, pose some risk for sexual dysfunction over time.
A decrease in white blood cells occurs in about 10% of those taking the drug. This is generally not serious unless infection accompanies it.
Other blood conditions can arise that are also potentially serious. Patients should be sure to inform the doctor if they have any sign of irregular heartbeats, sore throat, fever, easy bruising, or unusual bleeding.
Long-term therapy can cause osteoporosis in women, who should take preventive calcium and vitamin D supplements.
Children are at higher risk for behavioral problems.

Note: Citrus fruit, especially grapefruit, can increase carbamazepine's adverse effects and should be avoided by those taking this drug.

Phenytoin

Phenytoin (Dilantin) is effective for adults who have the following seizures or conditions:

Grand mal seizures.
Partial seizures.
Can be effective for people with head injuries who are at high risk for seizures.

This drug is not useful for the following seizures:

Petit mal seizures.
Myoclonic seizures.
Atonic seizures.

Side Effects. Side effects are sometimes difficult to control. Some people may develop a toxic response to normal doses, while others, such as those with alcoholism, may require higher doses to achieve benefits. As with any drug, side effects generally rely on dosage and duration. Using phenytoin in combination with newer add-on drugs can allow lower doses and may reduce some of the risks. Side effects include:

Excess body hair, eruptions and coarsening of the skin, and weight loss.
Staggering, lethargy, nausea, gum recession, depression, eye-muscle problems, anemia, and an increase in seizures can occur as a result of high doses.
The part of the brain that affects muscular stability can be damaged as a result of taking this drug in very high amounts or for long periods of time.
Liver damage may develop in rare cases.
Long-term therapy can cause osteoporosis in women, who should take preventive calcium and vitamin D supplements.

Valproate and Divalproex Sodium

Valproate (Depakene, valproic acid) and its delayed release form, divalproex sodium (Depakote), are anticonvulsants. They are used to prevent nearly all major seizures, including generalized seizures, some cases of juvenile myoclonic epilepsy, and seizure combinations.

General 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. General side effects include:

Stomach and intestinal problems are experienced by nearly half of patients after starting the drugs and may still occur after several years of use. Divalproex sodium (Depakote) has a lower risk for these side effects than valproate (Depakene).
Increased appetite with significant weight gain often becomes a problem.
Hand tremors, irritability, and hyperactivity in children are fairly common.
Temporary hair thinning and hair loss have occurred; taking zinc and selenium supplements may help reduce the effect.
Young girls may develop secondary male characteristics and premenopausal women are at increased risk for menstrual irregularities and polycystic ovaries due to elevated male hormones, which are reversible. (These side effects also appear in women using other antiepileptic drugs, but the risk from valproate appears to be higher.)
The drug poses a significant risk for birth defects. Valproate is the preferred drug for women taking oral contraceptives.
Studies are reporting symptoms of Parkinson's disease preceded by hearing loss in people who have taken it for more than a year, but they were reversible when the drug was withdrawn.

Toxic Side Effects

Cases of pancreatitis, a serious and even life-threatening inflammation in the pancreas, have been reported in children and adults taking valproate. (It is still very rare, however.)
Valproate and divalproex sodium are not usually recommended for young children because of an unusual, but potentially fatal, toxic effect on the liver. [See Table of symptoms.] It should be noted that this very rare effect is most likely to affect children under two years of age who have birth defects and are taking more than one antiseizure drug. Some physicians recommend monitoring of blood levels for liver function once before administering valproate or divalproex sodium, monthly during the first six months, and then periodically after that.
Children with epilepsy who take valproic acid may eventually develop some problems in the kidney, although according to a 2001 study, they are generally not significant.

Symptoms of Toxic Side Effects in Liver or Pancreas

Abdominal pain (liver or pancreas)

Nausea or vomiting (liver or pancreas)

Loss of appetite (liver or pancreas)

Lethargy

Acute confusion

Water retention

Easy bruising

Yellowish skin coloring

Phenobarbital

Phenobarbital (Luminal) is a barbiturate anticonvulsant and is often the initial drug prescribed for newborns and young children. It is used to prevent grand mal seizures or simple partial seizures, but is not useful and may even exacerbate complex partial seizures or petit mal seizures. Many physicians prefer to prescribe drugs that have fewer sedating effects than phenobarbital. On the other hand, phenobarbital has fewer toxic effects on other parts of the body than most antiepileptic drugs, and drug dependence is unusual, given the low doses used for patients with epilepsy.

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.
Some controversy has arisen over studies indicating that children taking phenobarbital score lower on intelligence tests, even for some months after going off the drug.

Primidone

Primidone (Mysoline) is converted in the body to phenobarbital, and so has the same benefits and adverse effects. It is reported that primidone is not as well-tolerated as phenobarbital. Some authorities even believe that primidone has no advantage over the other drug.

Ethosuximide

Ethosuximide (Zarontin) is used for petit mal (absence) when the patient has experienced no other type of seizures. Ethosuximide succeeds in abolishing petit mal seizures in 60% of patients and controls them in up to 90%. Use of this drug can cause stomach problems, dizziness, loss of coordination, and lethargy. In rare cases, it has caused severe and even fatal blood abnormalities. Periodic blood counts are recommended for patients taking this drug.

Clonazepam and Clobazam

Clonazepam (Klonopin) is recommended for myoclonic and atonic seizures that cannot be controlled by other drugs and may be useful in newborns in whom other drugs are ineffective. Although clonazepam can prevent generalized or partial seizures, patients generally develop tolerance to the drug, and seizures recur. Clobazam, a similar drug, is currently under investigation. It is inexpensive and effective in some patients not responsive to other drugs. Clobazam shares similar side effects with clonazepam, and like clonazepam, clobazam loses effectiveness within a few months.

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

Add-Ons or Secondary Medications

Since 1993, eight anti-epileptic agents have been introduced in the US that have been designed to interrupt specific processes leading to epileptic seizures. Presently, these drugs are generally used only in combination with other drugs. Many are being tested successfully as sole or initial therapy, however, and a 2001 article recommended that they even be considered for earlier use in the treatment cycle. All appear to offer some benefits, but as with standard antiseizure drugs, they also have troublesome side effects. Studies are underway to determine the advantages of one over the other. Unfortunately, most studies report only on seizure reduction and few studies assess other factors, such as how the drug affects quality of life.

Lamotrigine. Lamotrigine (Lamictal) is proving to be effective as add-on therapy, including for seizures that do not respond to standard medications. For instance, in a 2001 study, a combination of lamotrigine and valproate relieved seizures in 10 out of 17 people with intractable frontal lobe epilepsy. It is useful in treating partial and generalized seizures and possibly for Lennox-Gastaut syndrome and absence seizures in children.

It also may be safe and effective as single therapy for newly diagnosed epilepsy. Studies overseas have found it to be as effective as carbamazepine and phenytoin when it is used alone, and patients tolerate it better. Lamotrigine may be a good alternative for people who experience weight gain or other hormone-related side effects from valproate.

Lamotrigine may not have the adverse effects on sexual function in men as some other antiseizure agents have. The drug also appears to improve cholesterol levels and, according to a 2001 study, may have longer-lasting effectiveness than vigabatrin when prescribed in patients who have been diagnosed in childhood with severe epilepsy.

A rash occurs in 5% of patients; it may disappear in some patients who continue taking the drug, but in rare cases it can become very severe. The risk of the rash increases if the drug is started at too high a dose or if the patient is also taking valproic acid. Other side effects may include nausea, dizziness, blurred vision, and sleepiness. Some patients report severe insomnia. A rare but serious side effect is anticonvulsant hypersensitivity syndrome, which is characterized by fever, skin eruptions, abnormal lymph nodes, and liver damage.

Gabapentin. Gabapentin (Neurontin) is a particularly effective add-on drug for controlling complex partial seizures and secondarily generalized partial seizures but not at all effective for petit mal seizures. It may prove to be a useful add-on for children with partial seizures that do not respond to standard treatment and may be particularly beneficial for the elderly, who tend to take many other medications.

Its toxicity is low and side effects include sleepiness, headache, fatigue, and dizziness. Some weight gain has been reported. Gabapentin has no significant interactive effects when taken with other drugs. It has the added advantage of improving mood, which is independent from its effect on seizure control.

Topiramate. Topiramate (Topamax) is similar to phenytoin and carbamazepine and is effective and safe for a wide variety of seizures in adults and children, including partial and generalized tonic-clonic epilepsies. Studies are showing a 34% to 87% reduction in seizure frequency with some patients becoming seizure-free. It may even help some children who have Lennox-Gastaut syndrome. A 2000 study reported that it was safe even in infants with severe myoclonic epilepsy, and in half of these patients seizure frequency fell by more than 50%, with 22% of them experiencing a reduction greater than 75%.

Most side effects are mild to moderate and can be reduced or even prevented by beginning at low doses and increasing dosage gradually. The most common side effects of topiramate include: mood swings and behavioral problems, dizziness, fatigue, visual disturbances, tremor, impaired concentration, weight loss and diarrhea, and a higher risk for kidney stones.

Vigabatrin. Vigabatrin (Sabril) is a chemical called gamma-vinylGABA. It was designed to increase the brain levels of gamma aminobutyric acid (GABA), the enzyme that inhibits seizure activity. It is effective in the elderly and in children, including those with infantile spasms (West syndrome). According to a 2001 study on children, it achieved a high initial response (32%) but after five years only 1.8% of children were seizure-free. Studies have also reported that is not as effective as carbamazepine, although it is better tolerated.

Side effects include weight gain, drowsiness, and sleep disturbances. Between 10% and 30% of people on long-term treatment have developed irreversible visual disturbances, including reductions in acuity and color vision. Men are at higher risk for this side effect than are women. Further studies are needed to determine the extent and severity of this complication, particularly in children. There is a slight risk for depression or psychosis when vigabatrin is used as add-on therapy, and particularly if the drug is administered too quickly. These risks are far lower if the drug is used as sole therapy.

Felbamate. Felbamate (Felbatol) is an effective antiseizure drug. However, after reports of deaths from a serious blood condition known as aplastic anemia or from liver failure, felbamate is only recommended for severe epilepsy, such as Lennox-Gastaut syndrome, and only when other drugs fail.

Zonisamide. Zonisamide (Zonegran) is a unique agent that blocks sodium and calcium channels and may have nerve-protecting properties. Studies indicate it is often effective against infantile spasms and myoclonic seizures. In Japan it has been beneficial in cases of severe epilepsy that did not respond to other drugs. Zonisamide increases the risk for kidney stones, which can be reduced with increased fluid intake and citrate. Other side effects tend to decrease over time and include: dizziness, forgetfulness, headache, weight loss, and nausea.

Levetiracetam. Levetiracetam (Keppra) is known as a nootropic agent and has been approved for partial onset seizures. Nootropics enhance mental function under conditions of low-oxygen levels. Some experts believe that levetiracetam represents a significant advance. One advantage of levetiracetam is that the initial dose is often effective as the ongoing daily dose, simplifying the regimen for both the patient and the doctor. It has fewer drug interactions than other anti-epileptic agents. It is also proving to be beneficial for patients with myoclonic epilepsy. Piracetam (Nootropyl) is a similar agent that is not approved in the US.

Side effects occur mostly in the first month. They include: sleepiness and fatigue, muscle weakness and coordination difficulties, headache, flu symptoms, dizziness, behavioral abnormalities, and possible risk of a reduced white blood cell count and a higher rate of infections. Caution is advised for patients with kidney dysfunction.

Oxcarbazepine. Oxcarbazepine (Trileptal) is similar to phenytoin and carbamazepine but has fewer side effects. It has recently been approved as monotherapy for partial seizures in adults and as add-on treatments for children. Oxcarbazepine, for example, appears to have few interactions with other drugs and may be particularly useful for elderly patients, who often require additional medication.

Tiagabine. Tiagabine (Gabitril) is another drug with properties similar to phenytoin and carbamazepine, and is also showing promise.

Other Agents

Methsuximide. Methsuximide (Celontin), a drug similar to ethosuximide, may be suitable as an add-on treatment for intractable epilepsy in children without causing serious or permanent side effects.

Investigative GABA Enhancers. New GABA enhancers under investigation include ganaxolone, retigabine and pregabalin. They may prove to be more effective than the current group of agents with similar actions (vigabatrin, tiagabine, gabapentin and topiramate).

Older Drugs

Some older but less effective drugs may still play a role against epilepsy:

Acetazolamide (Diamox) is sometimes used against common types of seizures, but patients quickly develop a tolerance for it. Some experts suggest it still may be useful when drug interactions are a problem, when a rapid effect is required, or when an additional drug is needed for a short time.
Trimethadione (Tridione) is effective for petit mal seizures, but has very serious side effects, and its use is severely limited.

WHAT ARE THE SURGICAL TREATMENTS AND OTHER PROCEDURES FOR EPILEPSY?

Surgical techniques to remove injured brain tissue may be appropriate for many patients with epilepsy. The surgeon's goal is to remove only the damaged tissue in order to prevent seizures and to avoid healthy brain tissue. Surgical techniques for reaching these goals have improved significantly over the past decades due to advances in imaging and monitoring, new surgical techniques, and a better understanding of the brain and epilepsy.

Tests to Determine if Surgery Is Indicated

A number of tests using imaging and electroencephalography (EEG) can determine if surgery is an option.

The general approach is to first use long-term EEG monitoring to locate the brain tissue that triggers the epileptic event.
Advanced imaging techniques can provide valuable additional information. They include functional magnetic resonance imaging (fMRI), magnetoencephalography (MEG), positron emission tomography (PET) or single-photon emission computer tomography (SPECT) scans.
If the imaging tests indicate that more than one site is involved or their results conflict, then more invasive monitoring of the brain may be required, although the newer imaging tests are proving to be very accurate tools.

If such tests pinpoint a specific area in the brain as the location for seizures, then surgery is possible. The physician will also examine the test results to determine if the offending nerve cells perform vital functions and try to predict surgical outcome in certain cases.

Temporal Lobectomy

The most common surgical procedure for epilepsy is temporal lobectomy, which is performed when epilepsy occurs in the temporal lobe. (Surgery is not as successful in epilepsies that occur in the frontal lobe.) It involves removing small portions from the hippocampus, a portion of the brain that is involved in memory processing and is part of the limbic system, an emotional center.

Candidates. A typical candidates for this surgery is an adolescent or young adult with complex partial seizures that began between age five and 10, and although the seizures were often in remission, they eventually became intractable.

Young children may be more difficult candidates because they often have injured areas outside the temporal lobes. Nevertheless, surgery can be very successful in many children, even if more than one area is involved.

Success Rates. New imaging techniques are dramatically improving the success rates of temporal lobe surgery. A 2000 literature review estimates that more than 70% to 90% of appropriate patients remain seizure-free after temporal lobectomy. And in an important 2001 comparison of surgery versus medication, after a year, 58% of the surgical group were free of significant seizures versus 8% of those taking medications. The procedure also seems to be successful in the long term, but more studies are needed to determine relapse rates. Studies are further reporting that temporal lobe surgery improves quality of life and may even prolong survival. Cure is not always possible, however, and some patients may still experience some seizures. Experts recommend that medication be continued as necessary, even if seizures are very infrequent.

Effects on Mental Functioning. Although surgery on the left temporal lobe does not impair intelligence to any significant degree, studies on the effects of mental functioning and behavior are unclear:

One study reported that 10% of patients experienced significant decline in language abilities while another 9% reported significant improvement. In the study, about 16% reported improvement in nonverbal mental functions.
In another study of children, surgery improved behavior in 31% of the patients and mental function in 25%. (Detrimental changes in personality, emotions, or behavior are uncommon.)

In general, surgical effects on mental functioning and behavior depend on the extent and location of the surgical area.

Lesionectomy

Lesionectomy is a procedure that removes specifically abnormal tissues in certain conditions, such as the following:

Cavernous angiomas (abnormal clusters of blood vessels).
Low grade brain tumors.
Cortical dysplasias. (This is an abnormality in fetal development in which the normal migration of nerve cells is altered for some reason.)

This local surgery, which can cure the patient's epilepsy, has become possible with the advent of advanced imaging techniques such as MRI.

Other Surgeries

Other surgical procedures called hemispherectomy and corpus callosotomy offer hope for infants and young children with catastrophic seizures that occur in one or part of a hemisphere and for patients whose seizures are due to certain structural brain abnormalities or tumors.

Hemispherectomy. Hemispherectomy is the removal of half the brain leaving the deep structures intact. Surgery can take 12 hours and there is always some paralysis on one side of the body. There is also a small risk for hydrocephalus, coma, or even death. Quality of life is almost always improved, however, and the surgery does not reduce intelligence.

Corpus Callosotomy. Corpus callosotomy involves cutting the nerve fibers that connect one side of the brain to another. It does not remove brain tissue. It may be done in two stages. In the first there is a partial separation. If seizures continue, then the surgeon may perform a complete separation. This surgery can reduce (although not entirely stop) uncontrolled tonic clonic seizures. The procedure may end drop attacks in patients who suffer from secondary generalized epilepsy with diffuse brain damage, such as Lennox-Gastaut syndrome.

Vagus Nerve Stimulation

Vagus nerve stimulation (VNS), an electrical stimulation of the vagus nerve, is proving to be an effective treatment for epilepsy in many cases. The two vagus nerves are the longest nerves in the body. They run along each side of the neck, then down 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 Procedure. Electrical stimulation of the vagus nerve is proving to be an effective treatment for epilepsy in many cases:

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 three to five years and need to be removed and replaced by a simple surgical procedure.

Other devices that stimulate the thalamus (which relays pain, temperature, and touch sensations to the brain) are being tested. In one 2000 study, for example, this approach was promising for Lennox-Gastaut and for patients with generalized seizures.

Candidates. The American Academy of Neurology now recommends this procedure for the following:

Patients who are over 12 years old, and
Have partial seizures that do not respond to medication, and
Αre not appropriate surgical candidates.

Research also indicates that it may be effective and safe in children with intractable epilepsy for whom surgery is the only treatment option. It might also be an appropriate therapy for status epilepticus. Its effectiveness for generalized seizures is unknown as yet. It should be noted that vagal stimulation does not eliminate seizures in most patients and it is still somewhat invasive.

Success Rates. Studies are reporting that the procedure reduces seizures within four months by up to 50% and even more in many patients. Although long-term studies are not yet available, the benefits seem to hold for up to two years. In one study that followed patients for a year, the benefits of VNS appeared to even increase over time.

Complications. Vagal stimulation 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. Studies are reporting no serious adverse side effects, although the treatment may cause lung function deterioration in people with existing lung disease.

Experimental Procedures

Gamma Knife Surgery. A device called a gamma knife delivers very focused beams of radiation. Typically used for brain tumors, it is now under investigation for temporal lobe epilepsy and for seizures due to cavernous malformations.

WHAT LIFESTYLE MEASURES CAN HELP PREVENT EPILEPTIC SEIZURES?

The best preventive measure is to comply strictly with the drug regimen as prescribed. 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 the following have been known to trigger them:

Inadequate Sleep. Inadequate sleep can set off seizures in some people (although in others seizures occur during sleep.)

Food Allergies. One study indicated that food allergies might provoke some seizures in children who also have migraine headaches, hyperactive behavior, and abdominal pains. Parents should consult an allergist if they suspect foods or additives might be playing a role in such cases.

Alcohol and Smoking. Alcohol and smoking should be avoided, although light alcohol consumption does not appear to increase seizure activity in people who are not alcoholics or sensitive to alcohol.

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 in Japan among people who watched cartoons with rapidly fluctuating colors and quick flashes. The frequency of flashes per second is measured in hertz (Hz). Screens that emit a lower hertz (such as 50 Hz screens sold in Europe) are more likely to cause seizures in people with epilepsy than a higher-hertz screen (such as 100 Hz screens sold in the US).

Relaxation Techniques and Yoga

Some people with epilepsy have found that relaxation methods help reduce the severity of the attacks. Such methods include diaphragmatic rhythmic breathing, and meditation techniques.

Some small studies have reported significant benefits from the practice of yoga. For example, in one study, a system of mediation called Sahaja yoga changed EEG readings of brain waves and reduced seizures. Other studies report a 50% reduction in seizures and an overall decline in number of attacks per month. Still, well-controlled studies are needed to confirm these benefits. It should be noted that there have been some reports that deep breathing (a common relaxation technique) triggers seizures in certain people.

Dietary Measures

Fasting has been used to prevent seizures since ancient times; in fact, a 2000 study of epileptic mice suggests that a restricted caloric intake while on a balanced diet can lead to measurable seizure reduction among all age groups. In the 1920s, a high-fat, no-sugar, low protein diet, known as a ketogenic diet, was used to prevent seizures. It lost popularity after the introduction of antiepileptic drugs but is now proving to be effective with many children. [ See Box The Ketogenic Diet, below.]

The Ketogenic Diet

The diet is based on the premise that burning fat instead of carbohydrates causes an increase in ketones. Excess ketones (called 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 of the Ketogenic Diet. Studies are reporting significant reductions in seizures in up to 85% of children who are good candidates for the program, and in one study 32% were nearly seizure-free. In a 2000 study, more than 60% of children experienced a greater than 50% reduction in seizures even after the diet was discontinued. Many children also report significant improvements in attention and social functioning one year after starting the diet.

Candidates of the Ketogenic Diet. The Ketogenic Diet seems to be most helpful for children in the following groups:

Children aged one to 10 who suffer from Lennox-Gastaut syndrome.
Children who suffer from severe seizures associated with injury or birth defects.
Children with severe symptoms who do not respond to medications. Children younger than 12 have the best outcome, but some studies are suggesting the diet may be useful for motivated adults with severe epilepsy that does not respond to medication.
Possibly safe and effective in some infants with uncontrolled seizures.
Most studies report benefits from the diet on patients with generalized seizures but some patients with partial seizures may benefit from it.

Typical Ketogenic Diet. It should be strongly noted that this diet must be professionally monitored. Parents can endanger their children if they try the program on their own without consulting a physician or trained health expert. The child fasts for the first two or three days, then the diet is gradually introduced. The regimen uses small amounts of carbohydrates and large amounts of fats (80%), with very few proteins and no sugar. A typical meal is 35 g white fish, 73 g vegetables, and 42 g butter or cream. Vegetables may include celery, cucumbers, or asparagus, cauliflower, and spinach.

Challenges. The diet is difficult and presents some challenges. Most importantly, 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). (Still, between 50% and 75% of children are able to stay on it.)

Side Effects and Complications. The diet also has a number of side effects. To prevent serious side effects, children will require regular monitoring by a physician.

Kidney stones (the most severe common side effects, occurring in 10% of children). Patients should drink plenty of fluids. Oral citrate may be protective.
The fats recommended in the diet (usually butter and cream) raise cholesterol levels in most children. Some parents have replaced the cream with heart-healthy olive or flaxseed oils and have found no increase in seizures in their children. Parents should be sure to consult with their physician about any changes to the diet. One study suggested that the diet may have some adverse effects on the heart in some children, but the problems appear to resolve when the diet is stopped.
Lethargy.
Nausea.
Diarrhea or constipation.
Dehydration.
Recurrent infections.
Weight loss.
Bad body smell.
Behavioral and mental changes.

Emotional and Psychologic Support

Everyone who suffers from epilepsy or who has a child with epilepsy can benefit from support associations, which are free and available in most cities. [ See Where Else Can Help Be Obtained for Epilepsy, below.]

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.
Often children can be given the hope that they will outgrow the disorder.
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. Because of the risks for serious emotional consequences, psychological therapy may be beneficial and even necessary for some adults and children.

In one study cognitive behavioral therapy was helpful in lowering seizure rates in young people with juvenile myoclonic epilepsy. This approach offers structured counseling program that helps people change behaviors that can reduce risk factors, such as anxiety and insomnia, for seizures.

WHERE ELSE CAN HELP BE OBTAINED FOR EPILEPSY?

Epilepsy Foundation, 4351 Garden City Drive, Suite 500, Landover, MD 20785-7223. Call (800-332-1000) or on the Internet (http://www.epilepsyfoundation.org/ )
This is the best epilepsy organization; it provides many services and excellent information on specific issues.

American Epilepsy Society, 342 North Main St., W. Hartford, CT 06117-2507. Call (860-586-7505) Or on the Internet (http://www.aesnet.org/ )

International League Against Epilepsy
http://www.websciences.org/engel/ (Under Construction as of 12/6/01)

American Academy of Neurology, 1080 Montreal Avenue, St. Paul, MN 55116. Call (651-695-1940) or on the Internet (http://www.aan.com/ )
Web site offers good information and provides names of neurologists for specific locations.

National Institute of Neurological Disorders and Stroke, Communications and Public Liaisons, P.O. Box 5801, Bethesda, MD 20824. Call (301-496_5751) or (800-352-9424) or on the Internet (http://www.ninds.nih.gov/ )
A good resource for information about research and participation in clinical trials.

Epilepsy Information Service, Wakeforest University School of Medicine, Medical Center Blvd., Winston-Salem, NC 27157-1078. Call (800-642-0500) Or on the Internet (http://www.wfubmc.edu/neuro/disease/epilinfo.shtml )
The service offers information packages to patients and professionals on different seizure types, medications, latest research, and resources.

Epilepsy Foundation of New Jersey, 429 River View Plaza, Trenton, NJ 08611. Call (609-392-4900) or (800-EFNJ TIE) or on the Internet (http://www.efnj.com/ )

Medic Alert, 2323 Colorado Ave., Turlock, CA 95382. Call (888-633-4298) or on the Internet (http://www.medicalert.org )
This organization provides bracelets or neck chain emblems with critical personal medical information. Also keeps computerized medical records.

Useful Internet Sites

Massachusetts General Hospital Epilepsy Surgery unit
(http://neurosurgery.mgh.harvard.edu/epilepsy.htm )

Washington University in St. Louis. Excellent information base with good information on drugs. (http://www.neuro.wustl.edu/epilepsy/ )

A very comprehensive database of pediatric disorders compiled by a Canadian pediatrician. (http://www.icondata.com/health/pedbase/index.htm )

High Plains Epilepsy Association
(http://www.llano.net/efsp/index.htm )

Andrews Rieiter Epilepsy Research Program. A website that offers a good description of a psychotherapeutic and behavioral approach to managing epilepsy. (http://www.andrewsreiter.org/)

Site where parents exchange stories about children with epilepsy (http://www.geocities.com/HotSprings/1000/ )