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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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Add-on epilepsy treatment okayed for children
From Nursing, 6/1/03

LAMICTAL

Lamictal (lamotrigine) tablets have been approved as add-on therapy to treat partial seizures in children age 2 years and older. Previously, the drug was approved for adjunctive use in adults with partial seizures and for treating generalized seizures in children age 2 years and older with Lennox-Gastaut syndrome. Partial seizures are the most common form of epilepsy.

Approval of Lamictal for the new indication was based on a clinical study that demonstrated the drug's effectiveness as add-on therapy in children who were still having frequent partial seizures (at least four per month) despite optimal doses of other anticonvulsant drugs. In the 18-week study of 199 patients ages 2 to 16 years, children treated with Lamictal as adjunctive therapy experienced a 36% reduction in frequency of all partial seizures, versus only a 7% reduction in placebo groups. In 42% of children taking Lamictal, the frequency of partial seizures was cut in half; only 16% of those in the placebo group experienced this result. Children receiving Lamictal also experienced more days without seizures than those in the placebo group.

Adverse reactions associated with Lamictal in clinical trials included dizziness, ataxia, tremor, and nausea; most were mild or moderate in severity.

Lamictal is a product of GlaxoSmithKline.

Copyright Springhouse Corporation Jun 2003
Provided by ProQuest Information and Learning Company. All rights Reserved

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