Failure of ketogenic diet therapy in GLUT1 deficiency syndrome
YAC Bekker, DA Lambrechts, JS Verhoeven… - European Journal of …, 2019 - Elsevier
Purpose Epilepsy in GLUT1 deficiency syndrome is generally drug-resistant; ketogenic diet
(KD) therapy is the mainstay of therapy, as production of ketones provides the brain with an
alternative energy source, bypassing the defect in GLUT1. Failure of KD therapy and risk
factors for failure have been sparsely published. Methods We performed a retrospective
study of GLUT1DS patients with refractory epilepsy failing on KD therapy, to identify their
clinical characteristics. Results Failure of the ketogenic diet was due to KD inefficacy (poor …
(KD) therapy is the mainstay of therapy, as production of ketones provides the brain with an
alternative energy source, bypassing the defect in GLUT1. Failure of KD therapy and risk
factors for failure have been sparsely published. Methods We performed a retrospective
study of GLUT1DS patients with refractory epilepsy failing on KD therapy, to identify their
clinical characteristics. Results Failure of the ketogenic diet was due to KD inefficacy (poor …