Lennox-Gastaut syndrome (LGS) is a rare and often debilitating form of childhood-onset epilepsy. The syndrome is characterized by a triad of signs including multiple seizure types, moderate to severe cognitive impairment, and an abnormal EEG with slow spike- wave complexes. LGS constitutes between 1-4% of pediatric epilepsies and typically appears between the second and sixth year of life.

Some of the known causes of Lennox-Gastaut Syndrome include brain injury associated with pregnancy or birth (including asphyxia, low birth weight, and prematurity), severe brain infections (including encephalitis, meningitis and rubella), developmental malformations of the brain, or a history of infantile spasms. In 30-35 percent of cases, no cause can be found.

Most children are typically developing normal when first diagnosed, but then begin to lose skills, sometimes dramatically, in association with uncontrolled seizures. Young children with LGS may exhibit behavioral issues, personality disturbances, mood instability, and slowing of psychomotor development. Behavioral disturbances can include poor social skills and attention seeking behavior, which can be caused by the effects of the medication, difficulty interpreting information, or the electric disturbances in the brain. Some children with LGS are prone to develop non-convulsive status epilepticus (a continuous seizure state that is associated with a change in the child's level of awareness). This requires medical intervention to bring it to an end.
As children with Lennox-Gastaut syndrome grow older, the types of seizures change. In most cases, the drop-seizures (atonic) are replaced by partial, complex partial, and secondarily generalized convulsions. The uncontrolled seizures may become less of an issue as children grow, but impaired intellectual functioning and behavioral problems persist. Older children with LGS experience acute psychotic episodes, forms of psychosis with aggressiveness, character problems, and irritability. Cognitive problems include slowed reaction time and information processing. The main characteristics of mental deterioration are reported as apathy, memory disorders, impaired visuomotor speed, and perseverance. More information on adults with Lennox-Gastaut Syndrome can be found on the LGS Foundation's sister site, www.adultsandlgs.org.

School-age children are usually placed into a special education classroom or will receive home tutoring. Parents and caregivers may need to consider future options for their child, including legal guardianship, respite care, group home facilities, and/or day programs. For those individuals with LGS who do not have adequate neurological services in their state of residency, an out-of-state facility is suggested. Level 4 facilities (facilities which provide intensive neurological services, elaborate evaluations and treatment options) are typically recommended if the local neurologist or epileptologist does not have experience in treating Lennox-Gastaut syndrome. For a listing of epilepsy centers in the United States, visit the National Association of Epilepsy Centers.

The prognosis of Lennox-Gastaut syndrome can greatly vary from person to person. Complete recovery, with freedom of seizures and normal development, is unusual for a child with LGS. Although there is no cure, there is an array of treatment options available which can be further explored on our treatments page.

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