Lennox-Gastaut Syndrome

The history of Lennox-Gastaut syndrome (LGS) begins in 1939 when a slow (2.5 Hz) spike-and-wave pattern was described by Gibbs, Gibbs, and Lennox . LGS is a severe form of age related epilepsy that typically becomes apparent during infancy or early childhood. LGS belong to the classification of epileptic that are a group of disorders in which seizure activity leads to progressive cognitive dysfunction and constituting 1-4% of childhood epilepsies . Three necessary findings for the diagnosis of LGS are multiple generalized seizure types, a slow spike-and-wave pattern (less than 2.5 Hz) on EEG and cognitive Decline . The most common types of seizures associated with LGS are tonic and atonic seizures. A third type of seizure commonly is seen in Lennox- Gastaut syndrome is atypical absence seizures. Patients with LGS may develop normally before the onset of seizures, and then psychomotor regression begins. Because the seizures associated with LGS are usually refractory to treatment, intellectual impairment and learning problems may worsen over time. In approximately 70-80 percent of cases LGS has an identifiable cause and called symptomatic Lennox-Gastaut syndrome. Such as perinatal insult, cortical dysplasia, neurometabolic disorders T congenital infections, stroke, trauma, tuberous sclerosis, encephalitis or meningitis. In 17-30 percent of individuals a previous history of West syndrome is present. Lennox-Gastaut syndrome may also be classified as cryptogenic, in which the cause is unknown or cannot be determined after evaluation. Cryptogenic cases are presumed to result from an unidentified condition. Individuals with cryptogenic Lennox-Gastaut syndrome do not have a previous history of seizure activity, prior neurological problems or cognitive impairment before the development of the disorder. The prevalence of LGS is 0.1 per 1000 population for boys, versus 0.02 per 1000 population for girls. The annual incidence in children is estimated to be 2 per 100,000 children. A diagnosis of Lennox-Gastaut syndrome is usually made based upon a thorough clinical evaluation, a detailed patient history and a complete physical and neurological evaluation. EEG and MRI is necessary for diagnosis No specific therapy for LGS is effective in all cases and the disorder has proven particularly resistant to most anti-epileptic drugs(AEDs).Ketogenic diet, VNS therapy and epileptic surgery are other options.