Tracy Dixon-Salazar, PhD, Executive Director of the Lennox-Gastaut Syndrome (LGS) Foundation, provides an overview of the disease, the disorder’s unique resistance to treatment, and what the foundation is doing to help this patient community.
What Is Lennox-Gastaut Syndrome?
As Dr. Dixon-Salazar explains, LGS is a rare neurological condition that usually begins in childhood. It is characterized by multiple types of seizures and intellectual disability. LGS patients often have multiple seizures per day; sometimes hundreds of times that are treatment-resistant. The condition is not congenital. It can be caused by brain malformations, perinatal asphyxia, severe head injury, central nervous system infection, and inherited degenerative or metabolic conditions. In about one-third of cases, no cause can be determined.
Managing the Treatment-Resistant Seizures in Lennox-Gastaut Syndrome
According to Dr. Dixon-Salazar, the initial stage of LGS is especially frustrating. The nature of LGS means patients’ seizures get worse and more frequent over time. These seizures are also uniquely resistant to treatment. Many patients will try 10 or more drugs in their life and will not respond to any of them. With that said, there are a few FDA approved treatments for LGS (e.g., rufinamide, clobazam, cannabidiol) and even more for epilepsy. However, Dr. Dixon-Salazar points out that at least one treatment for LGS was FDA approved even though only 6% of the patient population receiving the drug were seizure-free at 2 year follow up.
Additionally, Dr. Dixon-Salazar notes that it is important to remember that even if a LGS patient does find a drug that reduces their seizures, the brain damage caused by a lifetime of daily seizures is severe and the intellectual disability that accompanies LGS creates a number of other concerns for caregivers.
How the Lennox-Gastaut Syndrome Foundation Helps LGS Patients and Families
The LGS Foundation has an online support group of 6000 members from 27 countries. This group allows parents of LGS patients to help each other by suggesting ways to make daily life easier and by spreading information about latest research, better ways to navigate the healthcare system, etc. The LGS Foundation also has a patient assistance program which provides financial assistance to LGS patients. Additionally, the foundation funds research and hosts community events. One program that Dr. Dixon-Salazar is particularly proud of is the “Siblings Rock Program” which provides resources for siblings of LGS patients, a group that has been previously overlooked.
Visit the foundation’s website at https://www.lgsfoundation.org/
To learn more about Lennox-Gastaut syndrome and other rare neurological disorders, visit checkrare.com/diseases/neurology-nervous-system-diseases/