Childhood epileptic encephalopathy (Lennox-Gastaut syndrome [LGS]) is a rare and
debilitating form of childhood-onset epilepsy. The syndrome is characterized by frequent
seizures and multiple seizure types, a resistance to medications or therapies, mental
retardation or regression, and an abnormal EEG with generalized slow spike-and-wave
discharges. LGS constitutes between 1-4% of pediatric epilepsies and typically appears
between the second and sixth year of life.
Some of the known causes 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.
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.
The prognosis of Lennox-Gastaut syndrome can greatly vary from person to person.
Complete recovery, with freedom of seizures and normal development, is very unusual for a
child with LGS. Although there is no cure, there is an array of treatment options available
including the ketogenic diet, vagus nerve stimulation therapy, surgery, and anti-epiletic
drugs (AED's).
For in-depth vocabulary pertaining to LGS, please click here.
This information provided by the LGS Foundation is designed to provide basic information. It is not intended, nor does it
constitute medical or other professional advice. Diagnosis and advice on LGS should be sought from a medical professional.
debilitating form of childhood-onset epilepsy. The syndrome is characterized by frequent
seizures and multiple seizure types, a resistance to medications or therapies, mental
retardation or regression, and an abnormal EEG with generalized slow spike-and-wave
discharges. LGS constitutes between 1-4% of pediatric epilepsies and typically appears
between the second and sixth year of life.
Some of the known causes 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.
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.
The prognosis of Lennox-Gastaut syndrome can greatly vary from person to person.
Complete recovery, with freedom of seizures and normal development, is very unusual for a
child with LGS. Although there is no cure, there is an array of treatment options available
including the ketogenic diet, vagus nerve stimulation therapy, surgery, and anti-epiletic
drugs (AED's).
For in-depth vocabulary pertaining to LGS, please click here.
This information provided by the LGS Foundation is designed to provide basic information. It is not intended, nor does it
constitute medical or other professional advice. Diagnosis and advice on LGS should be sought from a medical professional.
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About LGS
LGS Vocabulary
Types of Seizures
Treatments
Living with LGS
Community Forum
Treatments
Donate
Resources
Media
Events
Stories
About Us
Copyright LGS Foundation, Inc., 2009