Information on Lennox-Gastaut Syndrome
Fact Sheet on Lennox-Gastaut Syndrome
- Lennox-Gastaut syndrome is one of the most severe forms of epilepsy. It accounts for up to 10 percent of all cases of childhood epilepsy, with slightly more males than females affected. It usually develops in children between 1 and 8 years of age, with 3 the average age of onset.
- Children with Lennox-Gastaut syndrome experience delays in their development and as many as dozens
of different, mixed types of seizures a day. The most common seizure types associated with Lennox-Gastaut
syndrome are tonic, atonic ("drop attacks"), myoclonic and atypical absence seizures.
- Tonic: stiffening of the body, with the eyes rolling upwards, dilation of the pupils and shallow, irregular breathing.
- Atonic or "drop attacks": brief loss of muscle tone and consciousness, causing abrupt falls.
- Myoclonic: sudden muscle jerks.
- Atypical absence: staring spells.
- Approximately 50 percent of patients with Lennox-Gastaut syndrome experience status epilepticus, a potentially life-threatening condition that is characterized by either a prolonged seizure lasting 30 minutes or more or the occurrence of repeated seizures without return of consciousness between attacks. Status epilepticus can last for hours, days, or even months, and the physical effects include stupor, dementia, greatly impaired balance and, in some cases, death.
- There is no known cause for Lennox-Gastaut syndrome, although some cases are thought to be the
result of the following:
- Brain injury due to problems associated with pregnancy and birth, including prematurity, asphyxia (lack of oxygen), and/or low birth weight.
- Encephalitis, meningitis, and severe brain infections such as those caused by toxoplasmosis and rubella.
- Genetic brain disease, such as tuberous sclerosis and inherited metabolic disorders.
- Malformations of the brain that occur during development.
- Lennox-Gastaut syndrome seizures are resistant to most antiepileptic drugs and thus are difficult to control. There is no cure for the disorder, and complete recovery (absence of any seizure activity and normal development) is unusual.
"When Your Child Has Lennox-Gastaut Syndrome"
The Jackie and Alyssa Disante Story“She is a little miracle to us every day.” That’s how Jackie Disante describes her daughter, Alyssa, an 11-year-old with Lennox-Gastaut syndrome (LGS). A severe form of epilepsy that accounts for up to 10 percent of all childhood cases, LGS can, in some cases, be fatal.
Alyssa experienced her first seizure a day before her second birthday. “I was called at work and told that there was an emergency and that I needed to go home,” Jackie remembers. “That drive home was the worst half-hour of my life.”
Alyssa was taken to a hospital emergency room, where she was diagnosed as having a seizure disorder. Within a few weeks, she was experiencing about 80 to 100 seizures a day. “She either seized or she slept. We held her 24 hours a day.”
Alyssa suffered from drop seizures - severe seizures characterized by a brief loss of muscle tone and unconsciousness that causes the affected person to fall. “The drop seizures were the worst part; they were violent and came on without any warning,” says Jackie. Like many children with Lennox-Gastaut, Alyssa was required to wear a helmet, along with elbow and knee pads, to protect herself from serious injury.
Because Alyssa needed full-time care, Jackie was forced to quit her job to be with her child around the clock. While at home, her free time was spent researching her daughter’s condition.
“At the time, I didn’t realize that ‘seizure disorder’ meant ‘epilepsy,’” Jackie recalls. And Alyssa’s doctors still hadn’t confirmed her diagnosis. Frustrated, but driven, Jackie searched for whatever information she could find and sought a second opinion. “My first question to the neurologist in the initial meeting was, ‘Do you think Alyssa has Lennox-Gastaut?’ to which he immediately responded, ‘yes, absolutely.’”
At this point, Alyssa was taking 5 antiepileptic drugs (AEDs) a day, but those medications couldn’t control her seizures. The physician gradually weaned Alyssa off these medications, and tried a combination of 2 new ones instead. “With the new medications, her seizures lessened, but they still weren’t controlled,” Jackie says.
When Alyssa was 6, her physician enrolled her in a new research study investigating the use of TOPAMAX - an AED - to treat Lennox-Gastaut syndrome in children. “When Alyssa began taking TOPAMAX, we saw immediate improvement,” Jackie remembers. “Her seizures lessened, and now they usually only occur when she’s sick, or sometimes when she’s sleeping.”
Four years later, Alyssa’s seizures are still under control with a combination of TOPAMAX and a
traditional AED.
Individual results may vary. TOPAMAX is approved as add-on therapy for adults and children ages 2 to 16 with seizures associated with Lennox-Gastaut syndrome, partial-onset seizures, or primary, generalized tonic-clonic seizures.
When used in combination with traditional antiepileptic drugs in clinical trials, some side effects
occurred, but were generally temporary. The most common side effects associated with TOPAMAX in children
included excessive drowsiness, loss of appetite, fatigue, nervousness, poor concentration, weight
loss, aggressive reaction with memory difficulty. Safety and effectiveness in children younger than
2 have not been established. In adults, the most common types of side effects were sleepiness, dizziness,
poor coordination, speech difficulty, slowed thinking, blurred or double vision, memory difficulties
and changes in sensation.
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About TOPAMAX®
TOPAMAX® is approved as initial monotherapy in patients 2 years of age and older with partial-onset or primary generalized tonic-clonic seizures.
Safety and effectiveness in patients who were converted to monotherapy from a previous regimen of other anticonvulsant drugs have not been established in controlled trials.
TOPAMAX® is approved as add-on therapy for patients 2 years of age and older with primary generalized tonic-clonic seizures, partial-onset seizures, or seizures associated with Lennox-Gastaut syndrome.