Since its introduction in 1921, the ketogenic diet has been used primarily for children with difficult-to-control seizures, often with drops and tonic seizures, and with a generalized epilepsy on EEG. This is essentially what is seen in the diagnosis of Lennox-Gastaut syndrome . For years, Lennox-Gastaut syndrome has been one of the most common epilepsy syndromes cared for at ketogenic diet centers.
Despite that, there have been nearly no studies specifically looking at the response of children with Lennox-Gastaut syndrome to the diet to date. Most references are subsets of larger studies, in fact. A search on Medline for "ketogenic" and "Lennox-Gastaut syndrome" will find about 50 articles, but none of them have both these terms together in the title! A study at our center in which children were treated with the ketogenic diet in combination with either a glucose (placebo) or saccharin (treatment) solution, in order to prove the diet works in a randomized, blinded, controlled manner, did use Lennox-Gastaut syndrome as the criteria for being included. However, the casual reader may not have noticed this fact.
If the ketogenic diet has been used for Lennox-Gastaut syndrome for 90 years, why study it now? In 2011, Lennox-Gastaut syndrome is a hot topic. Last year, rufinamide (Banzel™) was approved by the FDA with an indication for Lennox-Gastaut syndrome, one of the first times in history a new drug came on the market already with that labeling. Just a few weeks ago, clobazam (Onfi™) was approved by the FDA, also for Lennox-Gastaut syndrome (http://www.epilepsy.com/medications/clobazam) . On November 19, 2011 the Lennox-Gastaut Foundation is having a large conference in Arlington, Virginia to discuss all of the new and upcoming treatment options for Lennox-Gastaut syndrome now available. It's an exciting time.
In order to provide useful information to parents of children with Lennox-Gastaut syndrome, Dr. Monica Lemmon at Johns Hopkins Hospital looked at how children do in two ways. First, she reviewed the historical published literature from 1989-present to find cases of Lennox-Gastaut syndrome in these articles and see how these children did. When information was missing or incomplete, she contacted the authors. Second, she reviewed our own experience here at Johns Hopkins Hospital since 1994. This study was presented on October 28, 2011 at the annual meeting of the Child Neurology Society in Savannah, Georgia.
What did she find? In short, the ketogenic diet works as well as all other patients combined (about half of children with at least a 50% reduction in seizures). In the literature, 88 of 189 (47%) children with LGS had >50% seizure reduction after 3-36 months of KD treatment. Thirty-one (16%) became seizure-free. At Johns Hopkins specifically, 71 children with Lennox-Gastaut syndrome were treated, and 51% had a >50% reduction in seizures after 6 months. Only 1 became seizure-free. Many were able to reduce anticonvulsants.
Is the ketogenic diet more effective for children with Lennox-Gastaut syndrome than other syndromes? Not necessarily, according to these results. Does it work? Absolutely, in about half of children, but seizure freedom is rare. Children with Lennox-Gastaut syndrome that have seizures impacting their quality of life should certainly consider dietary treatment. Echoing this interest, Eisai, Inc., the creators of the website "Living with LGS", have added additional information on the ketogenic diet to their website with my assistance. On November 18th, they will begin to offer free copies of my book to any families that register! If you do this and enjoy the book, please thank them for it!
Lemmon ME, Terao NN, Ng Y, Reisig W, Rubenstein JE, Kossoff EH. Efficacy of the Ketogenic Diet in Lennox-Gastaut Syndrome: A retrospective review of one institution's experience and summary of the literature. Presented at the Child Neurology Society annual meeting, October 2011.
Crumrine P. Management of seizures in Lennox-Gastaut syndrome. Paediatr Drugs 2011; 13: 107-118.
Glauser T, Kluger G, Sachdeo R, Krauss G, Perdomo C, Arroyo S. Rufinamide for generalized seizures associated with Lennox-Gastaut syndrome. Neurology 2008; 70: 1950-1958.
Freeman JM, Vining EP, Kossoff EH, Pyzik PL, Ye X, Goodman SN. A blinded, crossover study of the efficacy of the ketogenic diet. Epilepsia 2009; 50: 322-325.
Ng YT, Conry JA, Drummond R, Stolle J, Weinberg MA, on behalf of the OV-1012 Study Investigators. Randomized, phase III study results of clobazam in Lennox-Gastaut syndrome. Neurology 2011;77:1473-1481.