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Lennox-Gastaut syndrome

Lennox-Gastaut syndrome (LGS), also known as Lennox syndrome, is a difficult to treat form of childhood-onset epilepsy, that most often appears between the second and sixth year of life and is characterized by frequent seizures and different seizure types and is often accompanied by mental retardation and behavior problems. more...

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Characteristics

As a rule, the age of seizure onset in LGS is between two and six years old. However, some patients get their first seizure within the first two years of life or after the first eight. The syndrome shows clear parallels to West syndrome, enough to suggest a connection.

Daily multiple seizures are typical in LGS. Also typical is the broad range of seizures that can occur, larger than that of any other epileptic syndrome. The most frequently occurring seizure types are: tonic, which are often nocturnal (90%); the second most frequent are myoclonic seizures, which often occur when the patient is over-tired.

Atonic, atypical absence, complex partial, focalized and tonic-clonic seizures are also common. Additionally, about half of patients will suffer from status epilepticus, usually the nonconvulsive type, which is characterized by dizziness, apathy, and unresponsiveness. The seizures can cause sudden falling (or spasms in tonic, atonic and myoclonic episodes) and/or loss of balance, which is why patients often wear a helmet to prevent head injury.

In addition to daily multiple seizures of various types, children with LGS frequently have have arrested/slowed psychomotor development and behavior disorders. The most common type

The syndrome is also characterized by an interictal (between-seizures) EEG featuring slow spike-wave complexes.

Incidence and Prevalence

Approximately 5% of children with epilepsy have LGS, and is more common in males than females. Whereas some children seem perfectly normal prior to the development of seizures, others already had some form of epilepsy, such as West syndrome, which is seen in 20% of patients before (symptomatic) LGS. West syndrome is characterized by Blitz Nick Salaam seizures, and typically evolves into LGS in the second year of life.

Finland

According to a 1997 community-based retrospective study in the Helsinki metropolitan area and the province of Uusimaa, the annual incidence of both Lennox-Gastaut was 2 in 100,000 (0.002%) from 1975-1985.

United States

0.026% of all children in the Atlanta, Georgia metropolitan area were estimated to have LGS in 1997, which was defined as, "onset of multiple seizure types before age 11 years, with at least one seizure type resulting in falls, and an EEG demonstrating slow spike-wave complexes (<2.5 Hz)." The study concluded that LGS accounts for 4% of childhood epilepsies.

Read more at Wikipedia.org


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Lennox-Gastaut treatment
From Nurse Practitioner, 2/1/02

Adults and children (aged 2 to 16) who suffer from seizures associated with Lennox-Gastaut syndrome have a new treatment option. The FDA approved topiramate (Topamax) tablets and sprinkle capsules as an adjunctive treatment for Lennox-Gastaut syndrome.

Researchers who studied 98 patients with Lennox-Gastaut syndrome found that topiramate used in combination with other drugs results in reduced seizure severity and the number of "drop attacks." Subjects had experienced a minimum of 60 seizures during the month before the study started. Subjects continued to receive any previously prescribed anti-epileptic drugs and were randomized to receive either topiramate or placebo as adjunctive medication.

Some 52% of family members caring for individuals who received topiramate reported overall seizure improvement, compared with 28% of caregivers of patients receiving placebo. Excessive drowsiness, loss of appetite, fatigue, nervousness, difficulty with concentration and attention, weight loss, aggressive reaction, and memory difficulties were the most common adverse effects observed in children. Sleepiness, dizziness, poor coordination, speech difficulties, slowed thinking, blurred or double vision, memory difficulties, and changes in sensation were the most common adverse effects in adults; generally, these effects were temporary.

Some patients using topiramate experience a syndrome of acute myopia associated with secondary angle closure glaucoma. Symptoms include acute onset of decreased visual acuity and ocular pain. These symptoms typically occur within 1 month of topiramate initiation.

Medication Update alerts readers to new drug approvals and other timely drug information. Health care providers should not make clinical decisions from these announcements, as they are not comprehensive.

Copyright Springhouse Corporation Feb 2002
Provided by ProQuest Information and Learning Company. All rights Reserved

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