This condition is a rare presentation of epilepsy in children, first described in 1966 by Dr. Henri Gastaut. Another doctor from Boston, US, named Willian G. Lennox first described the EEG characteristics of this rare epilepsy.
Named after these two neurologists, this syndrome is characterized by multiple factors including intellectual impairment, characteristic EEG findings, and triad of multiple seizure types.
Epidemiology
LGS is accountable for 2 to 5 percent of all childhood epilepsies and 10 percent of instances of epilepsy occurring in children younger than five years old. The incidence of this syndrome is estimated to range between 0.1 and 0.28 per 100,000 individuals.
The estimated incidence in children is 2 per 100,000. The prevalence is approximately 26 per 100,000 individuals. LGS is more prevalent in men than in women. No radical disparities have been reported
As diffuse brain injury accounts for majority of the cases, LGS is more typically identified in children with intellectual and/or developmental issues.
Anatomy
Pathophysiology
Etiology
In 1/4th of cases, this syndrome has no explainable cause, but otherwise it could occur for many reasons.
So, it’s etiology is divided into 2 subtypes:
Idiopathic or Crytogenic Lennox Gastaut syndrome- Under this subtype, the onset is generally later and no underlying pathology has been identified. But recent genetic studies have identified de novo mutations in some genes including GABRB3, ALg13, CHD2, and SCN1A genes. The significance of these mutations in the development of Lennox Gastaut Syndrome is still unknown.
Symptomatic Lennox Gastaut syndrome- This subtype is usually caused due to diffuse cerebral injury, and it accounts for about 3/4th of the cases. Causes for the same include injuries to the frontal lobe, meningitis, encephalitis, tuberous sclerosis, brain malformations during development, metabolic causes, and birth injuries. Almost 30% of children with LGS have a history of West syndrome, resulting in a worse clinical course.
Genetics
Prognostic Factors
The overall outcome for this syndrome is poor. Within 10 years of diagnosis, the mortality rate is between 3%-7%. Most of these deaths could be due to accidents.
If this syndrome is accompanied by the West syndrome or infantile spasms, the outcome is generally worse, in both cognitive status and seizure control.
Idiopathic patients have fewer morbidities and less severe symptoms. As LGS seizures are uncontrolled, they can cause Sudden Unexpected Death in Epilepsy (SUDEP).
400-800 mg orally each day divided twice daily
Maintenance dose- increase the daily dose by 400-800 mg each day unless the 3200 mg dose for each day is acquired
It is not known if the drug is effective at the lowest dose
Dose Modification
In the case of renal impairment or hemodialysis, adjust the dose
For more than 1 year-
10 mg/kg each day orally divided twice daily
Maintenance dose- increase the dose by 10 mg/kg every alternate day
Give a maximum of 45 mg/kg each day divided twice daily
Do not exceed the dose of more than 3200 mg/day
It is not known if the drug is effective at the lowest dose
Dose Modification
In the case of renal impairment or hemodialysis, adjust the dose
Future Trends
Media Gallary
References
https://www.ncbi.nlm.nih.gov/books/NBK532965/
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This condition is a rare presentation of epilepsy in children, first described in 1966 by Dr. Henri Gastaut. Another doctor from Boston, US, named Willian G. Lennox first described the EEG characteristics of this rare epilepsy.
Named after these two neurologists, this syndrome is characterized by multiple factors including intellectual impairment, characteristic EEG findings, and triad of multiple seizure types.
LGS is accountable for 2 to 5 percent of all childhood epilepsies and 10 percent of instances of epilepsy occurring in children younger than five years old. The incidence of this syndrome is estimated to range between 0.1 and 0.28 per 100,000 individuals.
The estimated incidence in children is 2 per 100,000. The prevalence is approximately 26 per 100,000 individuals. LGS is more prevalent in men than in women. No radical disparities have been reported
As diffuse brain injury accounts for majority of the cases, LGS is more typically identified in children with intellectual and/or developmental issues.
In 1/4th of cases, this syndrome has no explainable cause, but otherwise it could occur for many reasons.
So, it’s etiology is divided into 2 subtypes:
Idiopathic or Crytogenic Lennox Gastaut syndrome- Under this subtype, the onset is generally later and no underlying pathology has been identified. But recent genetic studies have identified de novo mutations in some genes including GABRB3, ALg13, CHD2, and SCN1A genes. The significance of these mutations in the development of Lennox Gastaut Syndrome is still unknown.
Symptomatic Lennox Gastaut syndrome- This subtype is usually caused due to diffuse cerebral injury, and it accounts for about 3/4th of the cases. Causes for the same include injuries to the frontal lobe, meningitis, encephalitis, tuberous sclerosis, brain malformations during development, metabolic causes, and birth injuries. Almost 30% of children with LGS have a history of West syndrome, resulting in a worse clinical course.
The overall outcome for this syndrome is poor. Within 10 years of diagnosis, the mortality rate is between 3%-7%. Most of these deaths could be due to accidents.
If this syndrome is accompanied by the West syndrome or infantile spasms, the outcome is generally worse, in both cognitive status and seizure control.
Idiopathic patients have fewer morbidities and less severe symptoms. As LGS seizures are uncontrolled, they can cause Sudden Unexpected Death in Epilepsy (SUDEP).
400-800 mg orally each day divided twice daily
Maintenance dose- increase the daily dose by 400-800 mg each day unless the 3200 mg dose for each day is acquired
It is not known if the drug is effective at the lowest dose
Dose Modification
In the case of renal impairment or hemodialysis, adjust the dose
For more than 1 year-
10 mg/kg each day orally divided twice daily
Maintenance dose- increase the dose by 10 mg/kg every alternate day
Give a maximum of 45 mg/kg each day divided twice daily
Do not exceed the dose of more than 3200 mg/day
It is not known if the drug is effective at the lowest dose
Dose Modification
In the case of renal impairment or hemodialysis, adjust the dose
https://www.ncbi.nlm.nih.gov/books/NBK532965/
medtigo
Lennox Gastaut Syndrome
Updated :
July 25, 2022
This condition is a rare presentation of epilepsy in children, first described in 1966 by Dr. Henri Gastaut. Another doctor from Boston, US, named Willian G. Lennox first described the EEG characteristics of this rare epilepsy.
Named after these two neurologists, this syndrome is characterized by multiple factors including intellectual impairment, characteristic EEG findings, and triad of multiple seizure types.
LGS is accountable for 2 to 5 percent of all childhood epilepsies and 10 percent of instances of epilepsy occurring in children younger than five years old. The incidence of this syndrome is estimated to range between 0.1 and 0.28 per 100,000 individuals.
The estimated incidence in children is 2 per 100,000. The prevalence is approximately 26 per 100,000 individuals. LGS is more prevalent in men than in women. No radical disparities have been reported
As diffuse brain injury accounts for majority of the cases, LGS is more typically identified in children with intellectual and/or developmental issues.
In 1/4th of cases, this syndrome has no explainable cause, but otherwise it could occur for many reasons.
So, it’s etiology is divided into 2 subtypes:
Idiopathic or Crytogenic Lennox Gastaut syndrome- Under this subtype, the onset is generally later and no underlying pathology has been identified. But recent genetic studies have identified de novo mutations in some genes including GABRB3, ALg13, CHD2, and SCN1A genes. The significance of these mutations in the development of Lennox Gastaut Syndrome is still unknown.
Symptomatic Lennox Gastaut syndrome- This subtype is usually caused due to diffuse cerebral injury, and it accounts for about 3/4th of the cases. Causes for the same include injuries to the frontal lobe, meningitis, encephalitis, tuberous sclerosis, brain malformations during development, metabolic causes, and birth injuries. Almost 30% of children with LGS have a history of West syndrome, resulting in a worse clinical course.
The overall outcome for this syndrome is poor. Within 10 years of diagnosis, the mortality rate is between 3%-7%. Most of these deaths could be due to accidents.
If this syndrome is accompanied by the West syndrome or infantile spasms, the outcome is generally worse, in both cognitive status and seizure control.
Idiopathic patients have fewer morbidities and less severe symptoms. As LGS seizures are uncontrolled, they can cause Sudden Unexpected Death in Epilepsy (SUDEP).
https://www.ncbi.nlm.nih.gov/books/NBK532965/
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