Lennox-Gastaut Syndrome: Not Just a Pediatric Problem
Lennox-Gastaut Syndrome: Not Just a Pediatric Problem
In October 2011, the FDA approved the use of clobazam as an adjunctive treatment for Lennox-Gastaut syndrome (LGS), an epileptic encephalopathy that tends to be refractory to treatment. LGS generally manifests in childhood, but persists into adulthood. Although a few cases of LGS first presenting in adults have also been reported, because LGS most commonly manifests before the age of 8 years, it is often erroneously thought of as a pediatric syndrome. Historically, LGS has been diagnosed according to a triad of signs and symptoms, including the presence of multiple intractable seizure types, mental retardation with behavioral disturbances, and electroencephalography findings dominated by generalized, slow spike-wave complexes. Because the condition is so debilitating, and its associated mental and psychiatric disorders may worsen over time, many LGS patients may ultimately require long-term care services. Annals of Long-Term Care® (ALTC) had the opportunity to discuss LGS and its treatments, including clobazam, with Selim R. Benbadis, MD, director, Comprehensive Epilepsy Program, University of South Florida and Tampa General Hospital, FL, who has written extensively on this disorder and raised awareness that LGS should not be overlooked in adult patients.
ALTC: Do the presentation and clinical characteristics of LGS differ between adult and pediatric patients?
Dr. Benbadis: Not really. LGS begins in childhood (usually early), so that is when the diagnosis should be made. The problem is that it is called other things, using vague terms like “seizure disorder,” “mixed seizure disorder,” and “complex partial seizures.”
In your published works, you note that there is some controversy on how to precisely define LGS and that using the classic triad is imprecise in making the diagnosis. Please explain.
The definition and borders are imprecise because it is not a disease with a single cause, such as multiple sclerosis, tuberous sclerosis, or Parkinson’s disease. It is a syndrome, which means a constellation of signs and symptoms that occur together and can be due to different causes.
Please briefly describe the currently available agents to treat LGS, including first-line and second-line medications.
All antiepileptic drugs can and are used for LGS because it’s often difficult to control. Some have an official FDA indication for LGS, but that is not very important.
In the United States, clobazam is the newest approved treatment for LGS. How does this agent fit into the treatment regimen?
It is related to, but different from, classic benzodiazepines, which are very often used for LGS. As is often the case for new drugs, no generics are available and the drug can be costly, so insurance companies require that older benzodiazepines be tried first.
