Lennox Gastaut syndrome (LGS): Feature article
For this newsletter issue, we are featuring a rare yet severe form of childhood epilepsy, called Lennox-Gastaut Syndrome. We were fortunate enough to have Christina Saninocencio, the founder of the LGS Foundation answer our questions and tell us a bit more about all the incredible things her foundation does for individuals living with LGS and their families.
What is Lennox-Gastaut Syndrome (LGS)?
Lennox-Gastaut Syndrome is a rare and severe form of childhood-onset epilepsy that is characterized by frequent seizures of various types, moderate to severe cognitive impairment, and a slow spike-wave EEG pattern. The average age of onset is between 2 and 8 years of age and typically persists into adulthood. Unfortunately, LGS is considered one of the "catastrophic" epileptic encephalopathies.
What might cause it?
There are a number of causes of LGS. These include: brain malformations, injury during birth (such as hypoxic ischemic injury) or in utero (such as stroke), meningitis, and encephalitis. Those who have a history of infantile spasms are also at a risk to develop LGS. Due to advances in genetic testing over the past decade, particularly whole exome sequencing, many new genes have been identified as causes for LGS.
What are the most common symptoms?
Multiple seizure types, with the most common being tonic seizures and developmental delay are two of the three characteristics needed to diagnose LGS. The other is a specific pattern on the EEG called "slow spike-wave". LGS is typically very treatment resistant, and many individuals will continue to have refractory seizures despite polytherapy (multiple medications).
What treatments are available?
There are six FDA approved medications specifically indicated for LGS: Rufinamide (Banzel), clobazam (Onfi), Felbamate (felbatol), lamotrigine (Lamictal), topiramate (Topamax), and now cannabidiol (Epidiolex). Other anti-epileptic drugs are also used in the treatment of LGS. In addition, dietary therapy (i.e. Ketogenic Diet, modified Atkins diet), and surgical options may be considered.
What is the typical course for individuals with Lennox-Gastaut syndrome?
There is no cure for LGS, but no two cases of LGS are the same. However, it is not uncommon for individuals with LGS to be taking multiple anti-epileptic drugs at one time. As children with Lennox-Gastaut Syndrome grow older, types of seizures may change. In most cases, the drop-seizures (atonic) are replaced by partial, complex partial, and secondarily generalized convulsions. Those with LGS may need extra services due to the complexity of the disorder. Caregivers can develop stress and anxiety-related problems, partly because the seizures are unpredictable and frequent. The health-related quality of life of both the child and the adult caregivers can be impacted by this syndrome.
How is the Lennox-Gastaut Foundation helping individuals with Lennox-Gastaut and their families?
The LGS Foundation has a number of programs to help LGS families around the world. These include: a patient assistance program, up-to-date information on clinical trials, news, and events, local support groups / meet ups, online and telephone support, forums, a Facebook® page, and our largest program, our international conference, held every 18 months in the United States. In addition to these programs, the LGS Foundation also funds scientific and clinical research to help advance the understanding of the disorder.
The LGS Foundation also has a "wish" partnership with Give Kids the World. Each year, the LGS Foundation awards two LGS families an all-expense paid trip to the Give Kids the World resort in Orlando, Florida. The winners are chosen via a lottery between January 1 and Jan 15 every year from applications sent in before December 31 the previous year.
If you visit https://www.lgsfoundation.org/ you can read much more about LGS as well as the resources and activities that this incredible foundation offers its members.
