An unfortunate challenge: Ketogenic diet for the treatment of Lennox–Gastaut syndrome in tyrosinemia type 1. (July 2016)

• Record Type: Journal Article
• Authors: De Lucia, Silvana; Pichard, Samia; Ilea, Adina; Greneche, Marie-Odile; François, Laurent; Delanoë, Catherine; Schiff, Manuel; Auvin, Stéphane
• Is Part Of: European journal of paediatric neurology. Volume 20:Number 4(2016:Jul.)
• Page Start: 674
• Page End: 677
• Publication Date: 2016-07
• Subjects: Antiepileptic drugs -- Epilepsy -- Ketogenic diet -- Lennox–Gastaut syndrome -- Tyrosinemia type 1; Pediatric neurology -- Periodicals; Nervous System Diseases -- Periodicals; Child -- Periodicals; Infant -- Periodicals; Neurologie pédiatrique -- Périodiques; Pediatric neurology; Electronic journals; Periodicals; Electronic journals; 618.928
• DOI: 10.1016/j.ejpn.2016.02.015
• Languages: English
• ISSNs: 1090-3798

Abstract

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.