Epilepsy

Seizures and Syndromes
Epilepsy: An Overview

February  2012
Epilepsy is a neurological condition that makes people susceptible to seizures. A seizure is a change in sensation, awareness, or behavior brought about by a brief electrical disturbance in the brain.

Seizures vary from a momentary disruption of the senses, to short periods of unconsciousness or staring spells, to convulsions. Some people have just one type of seizure. Others have more than one type.

Although they look different, all seizures are caused by the same thing: a sudden change in how the cells of the brain send electrical signals to each other.

If you have epilepsy, you probably already know that it’s not a mental disorder. It can be caused by anything that affects the brain, including tumors and strokes. Sometimes epilepsy is inherited. Often, no cause can be found.

Epilepsy is generally not the kind of condition that gets worse with time. Most adults who have it can expect to live a normal life span.

Doctors treat epilepsy primarily with seizure-preventing medicines. Although seizure medications are not a cure, they control seizures in the majority of people with epilepsy.

Surgery, diet (primarily in children), or electrical stimulation of the vagus nerve, a large nerve leading into the brain, may be options if medications fail to control seizures. Several drugs (called antiepileptic or anticonvulsant drugs) are prescribed to prevent seizures. Many factors are involved in choosing the right seizure drug. The goal of treatment is to stop seizures without side effects from the medicines.

When the doctor has made a diagnosis of seizures or epilepsy, the next step is to select the best form of treatment. If the seizure was caused by an underlying correctable brain condition, surgery may stop seizures. If epilepsy — that is, a continuing tendency to have seizures — is diagnosed, the doctor will usually prescribe regular use of seizure-preventing medications. If drugs are not successful, other methods may be tried, including surgery, a special diet, complementary therapy or vagus nerve stimulation (VNS). The goal of all epilepsy treatment is to prevent further seizures, avoid side effects, and make it possible for people to lead active lives.

When the doctor has made a diagnosis of seizures or epilepsy, the next step is to select the best form of treatment. If the seizure was caused by an underlying correctable brain condition, surgery may stop seizures. If epilepsy — that is, a continuing tendency to have seizures — is diagnosed, the doctor will usually prescribe regular use of seizure-preventing medications. If drugs are not successful, other methods may be tried, including surgery, a special diet, complementary therapy or vagus nerve stimulation (VNS). The goal of all epilepsy treatment is to prevent further seizures, avoid side effects, and make it possible for people to lead active lives.

Syndromes

Classifying epilepsy by seizure type alone leaves out other important information about the patient and the episodes themselves. Classifying into syndromes takes a number of characteristics into account, including the type of seizure; typical EEG recordings; clinical features such as behavior during the seizure; the expected course of the disorder; precipitating features; expected response to treatment, and genetic factors.

Epileptic syndromes can be either idiopathic (of unknown cause) or symptomatic of underlying brain damage or disease. In general, idiopathic forms have a better prognosis in terms of both seizure control and eventual remission than do symptomatic forms.

Childhood epilepsy can be grouped into syndromes based on several factors, including the kind of seizures the child has, when they began, how easy or difficult they are to control, and whether the child has other neurological effects.

For a number of years, it has been noted that some children with epilepsy appear very similar to other children with epilepsy in terms of the age their seizures begin, their type of seizures, EEG findings, response to treatment, or prognosis. These children are labeled as having a specific epilepsy syndrome. New epilepsy syndromes are constantly being identified with common epilepsy syndromes listed below:

Childhood absence epilepsy (petit mal epilepsy). Accounts for 2-4 percent of epilepsy in children and begins between ages 3 and 10. Seizures are brief staring spells with associated distinctive spike and wave EEG pattern. Absence seizures tend to occur very frequently. Forty percent of children with this epilepsy syndrome will outgrow it or go into remission by their teenage years.

Juvenile myoclonic epilepsy. (Also called Janz’s syndrome, impulsive petit mal, myoclonic epilepsy of adolescence, and jerk epilepsy). Typically begins at puberty in otherwise healthy children. The first symptom is usually a generalized convulsion. These children may also have myoclonic seizures (jerking of the muscles) on awakening. Convulsions may also occur. Can be controlled with medication, but not likely to be outgrown.

Benign Rolandic epilepsy (also called benign partial epilepsy of childhood). Accounts for more than one third of epilepsy beginning in middle childhood between ages 3 and 13. Involves simple partial seizures affecting the face, causing drooling and inability to speak, which may be followed by a convulsion. Seizures typically occur at night and these children otherwise are normal and healthy. The prognosis is favorable with 95% of children outgrowing their seizures by age 15.

Infantile spasms (West Syndrome). Clusters of rapid myoclonic jerks causing a baby to double up and jerk forward with their arms either bent or extended. They have a distinctive EEG pattern called hypsarrhythmia. This epilepsy syndrome is time limited, starting in the first year of life. Many children with infantile spasms have associated developmental delay and may go on to develop other forms of epilepsy, such as Lennox-Gastaut syndrome. Treatment may include steroid hormones, ACTH and antiepileptic drugs.

Lennox-Gastaut syndrome. Difficult to treat epilepsy syndrome with mixed seizures — including absence, tonic seizures causing stiffening, often during sleep; drop attacks, convulsive seizures on wakening, and a distinctive EEG pattern. Begins between the ages of 1 and 8. Children often have developmental delay and mental subnormality.

Rasmussen’s syndrome (also known as Rasmussen’s encephalitis). Rare condition. Affects one half of the brain, producing seizures and affecting control of the opposite side of the body. Various treatments have been tried, including surgery to remove the affected half of the brain.

Landau-Kleffner syndrome. Also a rare disorder beginning between ages of 3 and 7. Produces seizures and affects speech. Children develop normal speech, and then slowly lose it. Simple partial and tonic clonic seizures. Treated with antiepileptic drugs to control seizures, and, possibly, steroids.

Temporal lobe epilepsy. Complex partial seizures and simple partial seizures. Generalized tonic clonic seizures may be part of this syndrome as well. Treated with medication, surgery, or, in some cases, with VNS therapy.

Frontal lobe epilepsy. May produce weakness or inability to use certain muscles, including those that govern speech. Frontal lobe seizures may involve thrashing movements during sleep, also stiffening with the head turned to one side and the arm rising into a brief frozen state. Some seizures may be dramatic and upsetting to others, with screaming, bicycling movements of the legs, running. Treatment is with medication, and, in some cases, surgery.

Reflex epilepsy. Seizures triggered by something in the environment. Photosensitivity (the tendency to have seizures in response to flashing lights, strobe lights, or alternating patterns of light and dark) most common type. Begins in childhood, is associated with absence epilepsy, and may disappear in adulthood.

Progressive myoclonic epilepsy. A rare, form of epilepsy with myoclonic (jerking) and tonic-clonic (grand mal) seizures. Children with this condition may have trouble with maintaining balance and experience rigid muscles. There is also a loss of mental ability. A gene for this disorder has recently been discovered.

Seizures and Fever

Febrile (fever-caused) seizures affect many American children between the ages of 3 months and 5 years. Febrile seizures are not the same as epilepsy, although they may be the first seizures that a child with epilepsy has.

Febrile seizures occur when a child’s temperature rises rapidly, usually to 102 degrees or higher. There is often a family history of febrile seizures; they are most common around 18 months of age and affect between 3 and 4 percent of all children. Thirty to forty percent of children who have a febrile seizure will have another one, but most children outgrow the tendency as they mature. About 3 percent of children with febrile seizures go on to develop epilepsy.

In children with epilepsy, fever along with some drugs, medications, and sleep deprivation may trigger seizures.

Seizures and other conditions

Having a seizure is a sign of an underlying condition in the brain. In many cases, it is the only sign of a brain disorder. In other cases, it may be only one of many symptoms.

Common brain conditions that may also be associated with seizures include tuberous sclerosis, cerebral palsy, mental retardation, tumors, autism, and neurofibromatosis.

Epilepsy associated with other brain disorders is usually treated in the same way as epilepsy from an unknown cause.

Treatment for Children

Childhood epilepsy is usually treated with seizure-preventing medicines called antiepileptic or anticonvulsant drugs. If the drugs don’t work, or the child has a lot of side effects, surgery or the ketogenic diet may be tried.

Childhood epilepsy is usually treated with seizure-preventing medicines called antiepileptic or anticonvulsant drugs.

If the drugs don’t work, or the child has a lot of side effects, surgery or the ketogenic diet may be tried.

If surgery is not an option, or the diet does not work, a new form of therapy called vagus nerve stimulation (VNS) may be tried.

Medication

Children take the same antiepilepsy meds as adults do. Medication may be prescribed as tablets, sprinkles, capsules or in syrup.

These drugs are designed to prevent seizures. Some are successful with a limited number of seizure types; others have a broader range of action. Wherever possible, doctors try to control seizures with one drug. Some children, however, may have to take more than one.

A child may respond so well to medication that no further seizures occur so long as the medication is taken regularly and an effective level is maintained in the child’s blood.

A Continuing Need

Not having seizures does not mean that the medication is no longer needed. Most patients require a minimum of several years of therapy. Always ask your doctor before stopping antiepileptic medication.

Taking only part of your medication or stopping medication abruptly can cause a serious increase in seizure activity.

Finding the Right Drug

The search for the best medication for any individual child may take a long time. Children, like adults, respond to medications in different ways. Several drugs and different combinations of drugs may have to be tried in an effort to get the seizures under control.

The goal of treatment is to achieve the greatest level of control, the lowest level of side effects, at the lowest possible dose.

Common side effects from antiepileptic drugs include fatigue, nausea, changes in vision, and weight gain.

Some side effects are linked to high dosages. Others are due individual sensitivity or allergic reaction. Some tend to happen when a new medication is started, but go away (or become less of a problem) as the child becomes accustomed to it.