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. 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.
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.
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. 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.
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.
Copyright LGS Foundation, Inc., 2009
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LGS Vocabulary
Types of Seizures
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Living with LGS
Community Forum
Treatments
Donate
Resources
Media
Events
Stories
About Us
