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Epilepsy

Description

An in-depth report on the types, causes, diagnosis, and treatment of epilepsy.

Outlook and Effects

General Outlook for Patients with Epilepsy

Most patients can control their seizures with a single drug and stop drug treatment completely after 2 seizureless years. In fact, the sooner patients achieve remission using an anti-epileptic drug (AED), the better their chances for remaining seizure-free in the future. If epilepsy is not effectively treated, and if the patient has continuing seizures, changes in the neurons may eventually cause intractable, or refractive, epilepsy. This type of epilepsy is hard to control. Early treatment is extremely important.

Effects of Epileptic Seizures on the Brain. Some studies have reported changes in brain structures in epileptic patients, but it is unclear if such changes are a cause or result of seizures. A reassuring 2003 study found no indication that seizures cause any progressive abnormalities in the brain. However, a 2005 study suggested that people with a history of epilepsy have a higher risk of later developing schizophrenia or schizophrenia-like psychosis.

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 (SE) is a serious, potentially life-threatening, condition that can lead to chronic epilepsy. It occurs in 100,000 - 150,000 people in the U.S. 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%. (This high mortality rate is most likely due to a high incidence of myoclonic SE in elderly adults after cardiac arrest. One study reported much lower mortality rates from SE when cardiac arrest in elderly epilepsy patients is excluded.)

The condition is 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 least 5 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 anti-epileptic medications (accounts for about a third of status epilepticus events)
• Abrupt withdrawal of certain anti-epileptic 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 resulted in higher disability after the stroke, particularly if these severe seizures occurred within a week of the stroke.
• Low blood sugar in people with diabetes

Survival Rates

Epileptic patients who are cured have a normal lifespan. Their long-term survival rates are 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. The specific cause of the seizure may also contribute to fatalities.

There is a very low risk for sudden death in patients with epilepsy. Although the causes of such events are not fully known, experts suspect heart arrhythmias in many cases. There is some evidence, in fact, that a malfunction in the autonomic nervous system (which controls heart rate) may be responsible for some of these deaths. Some researchers believe that temporal lobe surgery in appropriate patients may reduce the risk. Drugs that block arrhythmias may also be helpful in reducing this risk.

Effects of Epilepsy in Children

Chance for Recurrence After a First Seizure. According to a 2000 study, about 64% children with one seizure unrelated to fevers have another one, and nearly two-thirds who have a history of more than one seizure are likely to have more seizures. Researchers are trying to find ways of predicting which children have the best chances to become seizure free, and which ones will not. Studies suggest that the frequency of early seizures, not their total number or type, determines whether a child will develop intractable epilepsy.

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

The long-term outlook for children with idiopathic epilepsy (epilepsy of unknown causes) is very favorable. One study reported that 68 - 92% of these patients were seizure-free after 20 years. Another study reported that they had a survival rate no different from children without these seizures.

Children whose epilepsy is a result of a specific condition (for example, a head injury or neurologic disorder) have higher mortality rates than the normal population, but their lower survival rates are most often due to the underlying condition, not the epilepsy itself.

Side Effects of Medications and Withdrawal from Them. The drugs used for epilepsy can have distressing short- and long-term effects. Eventually, many children with epilepsy can go off medication. Children who tend to relapse after withdrawal from treatment usually have 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.

• 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 1, 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. 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 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. They were slightly more likely to be unemployed, unmarried, and childless compared to the general population.

Effects of Epilepsy in Adults

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 2 - 5 years
• A normal EEG reading
• A single-drug treatment

In one study of adult-onset epilepsy, it was discovered that after 1 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. One study found that IQ scores increased in adults with recurrent seizures during the trial period. A previous study yielded the opposite result, reporting that intelligence scores start declining with long duration of adult epilepsy. More research is needed in this area, as results have been contradictory.

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. Treatments can cause considerable physical effects, such osteoporosis and weight changes.

Emotional Consequence. About 25 - 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:

• 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 drugs may reduce this problem.

Effects on Female Fertility and Pregnancy. Epilepsy and its treatments can have adverse effects on female fertility and pregnancy.

Epilepsy and Pregnancy Studies have been conflicting on the effects of fertility from epilepsy, but most suggest that fertility rates among women with epilepsy are lower than among women in the general population. A number of factors, including anti-epileptic drugs (AEDs) or social factors, such as marriage at an older age, may contribute to this lower rate. Certain AEDs, particularly valproate, disrupt ovulation and menstruation by increasing male hormone levels and weight and causing polycystic ovaries. Effects of Epilepsy on the Pregnant Patient and the Fetus 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. Effects of Seizures. Isolated seizures do not appear to pose any adverse effects to the mother or the 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 on the Fetus. 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. 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 9 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 for Women Who Want to Become Pregnant A woman who wishes to become pregnant and has been seizure-free for 2 or more years may attempt to discontinue drugs under her doctor’s supervision. If she has not been seizure-free, she should continue medications but try to reduce them to a single drug, if possible. (Again under a doctor’s supervision.) Steps During Unplanned Pregnancy If a woman taking antiseizure medications has an unplanned pregnancy, there may be no point in switching medications right away, since the effects of the drugs last for 10 weeks. However, she should notify her doctor immediately. She should 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 Some types of anti-epileptic drugs (AEDs) can increase the risks for birth defects, especially when taken during the first trimester of pregnancy. Expert guidelines advise that pregnant women use the most effective medication for their type of epilepsy at the lowest dose possible to control seizures. They should also have their doctors take blood tests during pregnancy to monitor their drug levels. A 2006 study compared the chances of fetal death or serious birth defects for four commonly prescribed AEDs. The risk was significantly higher for valproate than the other drugs. (Birth defects included skull and limb deformities, and brain, heart, and lung problems.) In this study of 333 mother-child pairs, the following percentages of pregnancies resulted in birth defects depending on drug used: Valproate (20.3%) Phenytoin (10.7%) Carbamazepine (8.2%) Lamotrigine (1.0%) This study was small, and included only a few types of AEDs. In general, research indicates that 90% of women who take AEDs will give birth to healthy children. Still, doctors recommend that women of child-bearing age use a drug other than valproate if possible. 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. The risk increases to 20% when four drugs are used. Birth Defects Associated with Medication. The most common birth defects related to anti-epileptic drugs are: Cleft lip or palate (risks from lamotrigine, phenobarbital, phenytoin, valproate especially when taken during first trimester) Genital or urinary abnormalities (risk from most standard drugs) 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 women with epilepsy should talk with their doctor about taking a supplement of folic acid (5 mg) at least 3 months before conception 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 antiseizure drugs also cause a deficiency in vitamin K clotting factors that increases the risk for hemorrhage in the newborn. Treatment with vitamin K during the last month of pregnancy 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 (extremely high 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 birth defects. Also, if the mother was given phenobarbital or primidone while pregnant, the infant should be monitored for up to 8 months to see if withdrawal symptoms develop. Drug dosages will also need to be adjusted for the mother after delivery. Breastfeeding. Women on most AEDs can usually nurse their babies, since usually only a small amount of the drug enters the breast milk. The lowest levels are with phenytoin and valproate. (Ethosuximide and possibly levetiracetam are exceptions and should be avoided when a woman is breastfeeding. Women taking phenobarbital are also 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.

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.

Household Accidents . According to a 2006 study, the kitchen and bathroom are two of the most dangerous places for children with epilepsy. Parents should take precautions to prevent burning accidents from stoves and other heat sources. Children with epilepsy should never be left alone when bathing.

Driving and the Risk for Accidents. Being unable to drive is an extremely distressing and severe component of epilepsy. Drivers with well-controlled epilepsy are not at a high or unacceptable risk for automobile accidents. Uncontrolled epilepsy, however, poses a high risk. Needless to say, seizures can be very dangerous if they occur while a person is driving. Studies have reported that more than a fourth of drivers with uncontrolled epilepsy had a seizure-related accident at some time. One study found that over half of these accidents resulted in injuries to the patient or others. In spite of these events, 30% of the patients had driven within the past year, and most drove at least once a week.

Four factors help predict who may safely drive:

• A long duration between seizures. In one study, being seizure-free for 6 months reduced the risk for accidents by 85%, and being seizure-free for 1 year lowered the risk by 93%. State laws restricting driving in people with seizures vary from requiring seizure-free periods of 3 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
• Having recently 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 avoid deep and cloudy water (a clear swimming pool is best), and always swim with a knowledgeable, competent, and experienced companion or have a supervisor on site.

• Review Date: 11/15/2006
• Reviewed By: Harvey Simon, MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital

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