Family impact

The severity of the seizures, frequent injuries, developmental delays, and behavior problems associated with Lennox-Gastaut syndrome (LGS) take a large toll on even the strongest families. Attention must be paid to the psychosocial needs of the family, especially siblings. The proper educational setting also is important to help the patient with LGS to reach his or her maximal potential.

Because of the high rate of injuries associated with atonic and tonic seizures, some patients with LGS may need to wear a protective helmet, including a face guard to protect the forehead, nose, and teeth. Some patients will not tolerate a helmet with a face guard. Even if tolerated, such helmets often are uncomfortable and rarely are cosmetically acceptable.25

Goals

As for all epilepsy patients, the goals of treatment for patients with LGS are the best quality of life with the fewest seizures (none, if possible), the fewest side effects, and the fewest medications.

Options

The treatment options for children with LGS can be divided into three major groups:

• First-line treatments based on clinical experience or conventional wisdom:
• valproic acid
• benzodiazepines
• pyridoxine (vitamin B6)
• Suspected effective treatments based on open-label, uncontrolled studies:
• adrenocorticotropic hormone (ACTH)
• corticosteroids
• intravenous immunoglobulin
• vigabatrin
• zonisamide
• ketogenic diet
• corpus callosotomy
• vagus nerve stimulation
• Effective treatments based on double-blind, placebo-controlled studies:
• felbamate
• lamotrigine
• topiramate

Only options in the third group have been rigorously and scientifically evaluated and found to be effective and safe for specific seizure types in LGS patients. Because no single treatment in any of the three groups gives satisfactory relief for all or even a majority of patients with LGS, however, a combination of treatment modalities frequently is needed.48

Adapted from: Glauser TA and Morita DA. Lennox-Gastaut syndrome. In: Devinsky O and Westbrook LE, eds. Epilepsy and Developmental Disabilities. Boston: Butterworth-Heinemann; 2001;65–78.
With permission from Elsevier (www.elsevier.com).
Reviewed and revised May 2004 by Steven C. Schachter, MD, epilepsy.com Editorial Board.