[The ketogenic diet in German-speaking countries: update 2003].

Area of Science:

• Neurology
• Pediatrics
• Metabolic Disorders

Background:

• The ketogenic diet has a long history in treating drug-resistant childhood epilepsies.
• It is the primary treatment for GLUT1 deficiency syndrome and pyruvate-dehydrogenase-complex-deficiency.
• Despite proven efficacy, the ketogenic diet remains underutilized in Europe for pediatric epilepsy.

Purpose of the Study:

• To evaluate the efficacy and tolerability of the ketogenic diet in European children.
• To assess its role in treating refractory epilepsy, GLUT1 deficiency syndrome, and pyruvate-dehydrogenase-complex-deficiency.
• To provide data supporting standardized therapeutic guidelines for the ketogenic diet in Europe.

Main Methods:

• Retrospective analysis of 146 children treated with the ketogenic diet across Austria, Switzerland, and Germany.
• Standardized questionnaires were distributed to 13 neuropediatric departments.
• Data collected included indications, treatment effects, and adverse events.

Main Results:

• In refractory epilepsy (n=111), 8% achieved seizure freedom, with 23% experiencing >50% seizure reduction.
• All patients with GLUT1 deficiency syndrome (n=18) and pyruvate-dehydrogenase-complex-deficiency (n=15) showed clinical improvement, with 94% seizure control in GLUT1 deficiency.
• Overall patient compliance was high at 82%.

Conclusions:

• The ketogenic diet is a highly effective treatment for GLUT1 deficiency syndrome and pyruvate-dehydrogenase-complex-deficiency.
• For refractory epilepsy, the ketogenic diet demonstrated comparable efficacy to many anticonvulsants and was well-tolerated.
• The findings support the ketogenic diet as a valuable therapeutic option, encouraging the development of European treatment standards.