An unfortunate challenge: Ketogenic diet for the treatment of Lennox-Gastaut syndrome in Tyrosinemia type 1

• Published in: European journal of paediatric neurology 2016-07, Vol.20 (4), p.674-677
• Main Authors: De Lucia, Silvana, Pichard, Samia, Ilea, Adina, Greneche, Marie-Odile, François, Laurent, Delanoë, Catherine, Schiff, Manuel, Auvin, Stéphane, MD, PhD
• Format: Article
• Language: English
• Subjects: Antiepileptic drugs; Child, Preschool; Diet, Ketogenic - methods; Diet, Protein-Restricted - methods; Epilepsy; Female; Humans; Infant; Ketogenic diet; Lennox Gastaut Syndrome - complications; Lennox Gastaut Syndrome - diet therapy; Lennox–Gastaut syndrome; Neurology; Pediatrics; Tyrosinemia type 1; Tyrosinemias - complications; Tyrosinemias - diet therapy
• ISSN: 1090-3798; 1532-2130
• DOI: 10.1016/j.ejpn.2016.02.015

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 aminoaciopathies. 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.