Even though it was 21 years ago, I vividly remember the first time I learned about Lennox-Gastaut
Syndrome (LGS). My daughter was 3 years old, had been suffering uncontrolled seizures for a few months, and I was frantically searching for any clue as to what was going on with her. I happened upon Dr. John Freeman’s book Seizures and Epilepsy in Childhood, which contained a chapter on the catastrophic epilepsies of childhood (that deserves an award for the WORST TITLE EVER!). As I read about LGS, I remember that some features of the syndrome fit with our daughter, Savannah, but others didn’t. Could she have LGS? I didn’t know. But what I did know, and what I can still remember feeling even now, was that the LGS definition written there scared the pants off me. One sentence in particular stuck with me. It went something like this: ‘kids with LGS continue to have seizures, regress developmentally over time, and live all of their lives dependent on others for their care.’ When she was finally diagnosed with LGS at age 5, my worst fears had come true.
Over the next 10 or so years, LGS took on a number of new meanings for us. There was Loathsome Gruesome Seizures (LGS). And Lousy Grisly Syndrome (LGS). And of course we can’t forget Lord, Gimme Sleep and Let’s Get Sweets (LGS). Chocolate was and is my friend! Of course these are the G-rated versions (I’ll leave the others to your imagination!). When I look at the description of LGS now, 21 years into our epilepsy journey, no wonder it scared the pants off me. Here it the definition of LGS in its 2017:
LGS is a rare and severe epileptic encephalopathy. Onset of LGS typically occurs before the age of 8 years, with peak occurrence between 3 and 5 years of age. Approximately 1% to 4% of all children with seizures have LGS and the prognosis for these patients is typically poor. Only 10% of patients will eventually achieve seizure remission while the remaining 90% of patients will continue to have life-long, treatment resistant seizures.
Due to a varied clinical presentation of LGS, diagnosis may be challenging. The triad of clinical signs required for diagnosis includes: multiple seizure types, moderate to severe intellectual impairment, and a presence of a generalized slow-spike and wave on an electroencephalogram (EEG). Accurate diagnosis of LGS is essential but may be difficult for a number of reasons, including multiple etiologies, a lack of specific biomarkers that distinguish LGS from other forms of epilepsy, a heterogeneous clinical presentation, and an EEG that may change over time.
What parent or loved one wouldn’t be fearful of that! But with all our years of anger, frustration, and fear (totally merited by the way, LGS is HORRIFIC!), we also found ways to see some good. Savannah is beautiful, kind, spunky, silly, and caring. She brings so much joy to our lives, as so many LGS kids do in their families and communities. Today, with all bad, LGS also means Lovely Genuine Savannah to us. We couldn’t imagine our lives without her, and wouldn’t want to.
As you read this new blog started by the LGS Foundation, and the many blog posts that will come, we hope you will find it informative, supportive, and hopeful. Especially hopeful! With all the pain we must endure at the hands of LGS, we must also find some good. Welcome! And until the next blog . . .
Tracy Dixon-Salazar is the Director of Research and Strategy at the LGS Foundation and an Independent Consultant. Her daughter, Savannah, has been living with LGS for more than 20 years.