Lennox-Gastaut Syndrome (LGS)
- Lennox-Gastaut Syndrome (LGS) designates a type of epilepsy with multiple different types of seizures.
- Intellectual development is usually, but not always, impaired.
- In about a quarter of children, no cause can be identified.
- Seizures usually don't respond to seizure medications.
- Accounts for only 2 to 5 percent of childhood epilepsies.
- Usually LGS persists through childhood and adolesence to adult years.
What is Lennox-Gastaut syndrome? The Lennox-Gastaut syndrome (LGS) is a type of epilepsy with multiple different types of seizures, particularly tonic (stiffening) and atonic (drop) seizures. Intellectual development is usually, but not always, impaired. The EEG shows a classic pattern of background slowing and spike-wave bursts at frequencies less than 2.5 per second. The cause of the disorder is unknown in 1 out of 4 children.
Tell me moreLennox-Gastaut Syndrome (LGS) accounts for only 2 to 5% of childhood epilepsies. Yet children with this type of epilepsy are well-known to both the pediatric and adult neurologist, because their seizures are hard to control and they will need life-long treatment. The intellectual and behavioral problems add to the complexity of this syndrome and the difficulties in managing life with LGS.
This epilepsy syndrome usually persists into the adult years, (changing some in its presentation with age) requiring familiarity by all health care professionals.
In the last few years, several new treatments have emerged. While none are a cure for LGS, this is good news for a group of children that historically has had few good treatment options.
How do you treat LGS? Treatment is difficult, because the seizures often don't respond to seizure medications or AEDs. The intellectual changes do not respond to any currently available medicine or treatment either.
Partial relief of seizures, and also falls and injuries from seizures, may be obtained by valproic acid, lamotrigine, topiramate, felbamate, clonazepam, rufinamide, clobazam and occasionally other medications.
Rescue therapies, or treatments that can be given to help stop or shorten clusters or seizures is an important part of seizure care for people with LGS.
Stimulation of the vagus nerve in the neck, with an implanted pacemaker (VNS Therapy) sometimes improves seizures in people with LGS.
Dietary therapies have also been very helpful in some people.
An operation to separate the two halves of the brain, called corpus callosum surgery, may reduce seizures and injuries, but obviously is a big undertaking.
Summary:LGS continues to present great challenges to children and adults with the syndrome, their families and their caregivers. Much more research is needed to identify better therapies of all types.
New advances in seizure detection, recording and alerting as well as other safety and protection devices are avenues that need further attention by families, health care providers, and researchers. These types of treatments and services can improve safety management and quality of life for people with LGS and their caregivers.
Who develops LGS? The prevalence of LGS or how many people have LGS at any point in time is about 0.26 per 1000. This means that 26 out of 100,000 people are living with LGS yearly. When only childhood epilepsies are considered, 1 out of 10 children with seizures beginning in the first 5 years of their life have LGS. It is more common in boys than girls.
Seizures usually begin in children at 26 to 28 months old with the majority starting before 7 years old.
Children who have other developmental or intellectual problems have a greater chance of being diagnosed with LGS.
Up to 17% of adults with cognitive problems who live in supported facilities and are unable to live independently have LGS.
The diagnosis of LGS can be difficult to make since it may take many years for different symptoms to develop. It is hard to know for sure how many people are affected at different ages.
No racial differences have been documented.
Authored by: Patricia O. Shafer RN, MN | Elaine Kiriakopoulos, MD, MSc | Joseph I. Sirven MD on 11/2014
Reviewed by: Joseph I. Sirven, MD | Patricia O. Shafer, RN, MN on 3/2014