Lennox-Gastaut syndrome (LGS) is a rare and severe form of epilepsy, accounting for up to 4 percent of all childhood epilepsy cases. LGS is characterized by three criteria: multiple seizure types, impairment of cognitive function, and a slow spike-and-wave pattern on an electroencephalogram (EEG)
According to the National Institute of Neurological Disorders and Stroke, LGS seizures usually begin before 4 years of age. Seizure types vary among patients, and can include tonic (stiffening of the body, upward deviation of the eyes, dilation of the pupils, and altered respiratory patterns), atonic (brief loss of muscle tone and consciousness, causing abrupt falls), atypical absence (staring spells), and myoclonic (sudden muscle jerks) seizures. There may be periods of frequent seizures mixed with brief, relatively seizure-free periods.
Most children with Lennox-Gastaut syndrome experience some degree of impaired intellectual functioning or information processing, along with developmental delays and behavioral disturbances. Lennox-Gastaut syndrome can be caused by brain malformations, perinatal asphyxia, severe head injury, central nervous system infection, or inherited degenerative or metabolic conditions. In 30-35 percent of cases, no cause can determined.
Treatment for Lennox-Gastaut syndrome includes anti-epileptic medications such as valproate, lamotrigine, felbamate, or topiramate. There is usually no single drug that will control seizures. Children who improve initially may later show tolerance to a drug or have uncontrollable seizures. Although I'm usually skeptical about claims of dietary treatments, I am impressed by the growing body of evidence pointing to the efficacy of a ketogenic diet (some form of low-carb regimen) as an adjunct to more conventional treatments. Write Japanese researchers Ito and Oquni:
Since the 1920s, a ketogenic diet, of low-carbohydrate, adequate-protein and high-fat content, has been used for the treatment of intractable childhood epilepsy. A decade ago this diet was tried as a last resort in the treatment of intractable epilepsy. However, recent advances in ketogenic diet have enabled it to become more commonly used worldwide even early in the course of epilepsy. Two less-restrictive ketogenic diets, namely, the modified Atkins diet and low-glycemic-index treatment, have been developed. These diets allow the patients and their families to choose a more liberal menu. Furthermore, a randomized controlled trial found that the ketogenic diet has a significant benefit, which strengthens the supportive evidence.
Caring for a child with a disability is never easy on a family, and the challenges of LGS can have a major effect on family dynamics. I learned just this week that a new video in the educational series “Navigating the Storm” has been launched on the "Living with LGS" website at www.livingwithlgs.com. The video's producers hope to help parents and caregivers anticipate and more effectively cope with family challenges. The educational website also provides a range of resources about LGS, including references on ketogenic diet research.
For More Information:
Lemmon ME, Kossoff EH. New treatment options for Lennox-Gastaut Syndrome. Curr Treat Options Neurol. 2013 Mar 8. [Epub ahead of print] http://link.springer.com/article/10.1007/s11940-013-0223-z
Ferrie CD, Patel A. Treatment of Lennox-Gastaut Syndrome (LGS). Eur J Paediatr Neurol. 2009 Nov;13(6):493-504. Epub 2009 Feb 10.http://www.ejpn-journal.com/article/S1090-3798%2809%2900002-6/abstract
Ito S, Oguni H. [Ketogenic diet for intractable childhood epilepsy; as an early option as well as a last resort]. Brain Nerve. 2011 Apr;63(4):393-400. [Article in Japanese]