Learn the Basics: Lennox-Gastaut Syndrome (LGS) 101

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Finding out your loved one has epilepsy is something most people never imagine. Knowing what to do next is what matters and that’s why education is key.

Lennox-Gastaut syndrome (LGS), a form of epilepsy often characterized by multiple types of seizures, is a rare condition, accounting for only two to five percent of childhood epilepsies. When LGS is diagnosed, it requires life-long treatment starting at a young age.

The onset of Lennox-Gastaut syndrome usually occurs before age eight. Additionally, the first clinical signs of LGS can begin with one or more seizure typesmost commonly, including:

Atonic: Known as drop attacks because they cause head nods, loss of posture, and sagging at the knees.
Tonic: Also known as drop attacks, and characterized by muscle tightening (this can happen to a specific set of muscles, or most muscle groups at once), eyes rolling back, and pupil dilation. Doctors will not diagnose LGS if a child doesn’t have this type of seizure. Tonic and atonic seizures can both result in unexplained falls, with possible injury.
Atypical absence: This seizure is usually characterized by staring, pauses in activity, and a lack of response. Symptoms may also include involuntary, repetitive behavior e.g. eye blinking and/or slight jerking of the lips.

LGS is often hard to diagnose since it is not identifiable by simple genetic tests or one single symptom. Therefore, physicians need to make the diagnosis based on a combination of clinical and EEG results. The classic LGS diagnostic criteria consist of three features, which may or may not appear all at once, including:

Multiple seizure types
Cognitive impairment or developmental delays
A slow spike-wave EEG pattern

“The specific causes and symptoms of LGS vary by patient. In fact, the cause of LGS is unknown in one out of four children with the condition,” said James W. Wheless, MD, Professor and Chief of Pediatric Neurology, The University of Tennessee Health Science Center, Director, Le Bonheur Comprehensive Epilepsy Program & Neuroscience Institute, Le Bonheur Children’s Hospital, Memphis, TN. “It’s important to see a doctor if you recognize any different behavior in your child.”

Some causes of LGS can include history of infantile spasms, genetic disorders such as tuberous sclerosis or injuries affecting the brain, such as lack of oxygen. Although 20 to 30 percent of children with LGS develop normally prior to the onset of symptoms, cognitive impairment (the inability to remember things, learn new things, concentrate, or make simple decisions) will likely appear during the progression of LGS.

It’s important to find the right resources and see a physician to get your child tested if he or she is showing signs and symptoms of LGS. For more information, visit livingwithlgs.com.