An unfortunate challenge: Ketogenic diet for the treatment of Lennox–Gastaut syndrome in tyrosinemia type 1. (July 2016) - De Lucia, Silvana
Abstract: The ketogenic diet is an evidence-based treatment for resistant epilepsy including Lennox–Gastaut syndrome. This diet is based on low carbohydrate-high fat intakes. Dietary treatment is also therapeutic for inborn errors of metabolism such as aminoacdiopathies. We report a child with both Lennox–Gastaut syndrome and tyrosinemia type 1. This epilepsy syndrome resulted form a porencephalic cyst secondary to brain abscesses that occurred during the management of malnutrition due to untreated tyrosinemia type 1. We used a ketogenic diet as treatment for Lennox–Gastaut syndrome taking into account dietary requirements for tyrosinemia type 1. The patient was transiently responder during a 6-month period. This report illustrates that ketogenic diet remains a therapeutic option even when additional dietary requirements are needed. Highlights: Ketogenic diet can be conducted in a patient with an additional dietary requirement. Ketogenic diet should not be considered as a last chance treatment even in more complex situation. Ketogenic diet can be helpful in the management of Lennox–Gastaut syndrome.