Abstract
Lennox-Gastaut syndrome is one of the rare childhood-onset epileptic encephalopathies, characterized by multiple type seizure disorder, the typical pattern on electroencephalogram and intellectual disability. Tonic-type seizures are most commonly seen in these patients. Behavioral disturbances and cognitive decline are gradual-onset and last long after the first episode of epileptiform activity. In most cases, there is some identifiable cause that has led to the clinical presentation of the patient. Various pharmacological and surgical procedures have been proposed for the treatment of Lennox-Gastaut syndrome and many more to come in the very near future to overcome the drug resistance and to avoid the patient forming a life-long dependency.
Highlights
BackgroundLennox-Gastaut syndrome (LGS) is one of the eight syndromes under epileptic encephalopathy, as reported by the International League Against Epilepsy (ILAE) task force [1,2]
The clinical picture of LGS is the triad of antiepileptic-resistant seizure disorder, characteristic abnormality on electroencephalogram (EEG), and cognitive dysfunction
Lennox-Gastaut syndrome is a typical childhood-onset, severe epileptic encephalopathy associated with a serious intellectual disability in 20%-60% patients at the time of the onset of seizures, the proportion of which will increase to 75%-95% at five years after the onset of seizures
Summary
Lennox-Gastaut syndrome (LGS) is one of the eight syndromes under epileptic encephalopathy, as reported by the International League Against Epilepsy (ILAE) task force [1,2]. Lennox-Gastaut syndrome was first named by Lennox as “Petit mal variant,” which was later named after him in 1966 by Gastaut and his coworkers as Lennox syndrome This was referred to as a childhood-onset tonic and the absence type of seizure [3]. Lennox-Gastaut syndrome is a typical childhood-onset, severe epileptic encephalopathy associated with a serious intellectual disability in 20%-60% patients at the time of the onset of seizures, the proportion of which will increase to 75%-95% at five years after the onset of seizures. The prevalence of Lennox-Gastaut syndrome is 1%-2% of the epileptic patients and 1%-10% (mean 4%) of the childhood epilepsies [3]. Lennox-Gastaut was once defined as any multiple seizure type epilepsy in children less than 11 years old. The causes of Lennox-Gastaut syndrome is divided into two broader groups [3,5]:
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