Neuropediatrics 2015; 46 - PS02-17
DOI: 10.1055/s-0035-1550729

Modified Atkins Diet, an Alternative Treatment of Glucose Transporter Type 1 Deficiency Syndrome

G. Illing 1, A. Wiemer-Kruel 1, T. Bast 1
  • 1Epilepsiezentrum Kork, Kehl-Kork, Germany

Aims: Compared with the conventional ketogenic diet (KD), the modified Atkins diet (MAD) is an easier to realize, fat enriched (∼ KD 1:1) diet without restriction of protein and calories. So far, the KD is the treatment of choice in cases of Glucose transporter 1 deficiency syndrome (GLUT1DS).

Methods: We report the case of 10 patients (five females and five males; age: 4–16 years, median: 8.6 years) with genetically proven GLUT1DS who were primarily treated with MAD. Of the 10 patients, 9 had active epilepsy and 1 had a paroxysmal movement disorder. The MAD was stopped in two patients because of GTCS. Of the 10 patients, 1 patient refused the MAD completely. One girl switched to classical KD after 21 months in spite of seizure reduction, but the noncompliance did not allow for satisfying, better effects than under MAD. Six children are still treated with an MAD. We evaluated seizure frequency, cognitive development, movement disorders, and behavior.

Results: Three of the six patients became seizure free and one of six experienced a seizure reduction of > 90%. The isolated movement disorder resolved in one of six patients and three of six patients showed a sustaining EEG normalization. AED were completely stopped in two of six leading to a reappearance of interictal spikes in one. A patient with additional focal cortical dysplasia experienced seizure deterioration after a short period of improvement and switching to a KD war refused. Coordination, cognition, and/or behavior were found to be improved in all patients.

Patients responding to the therapy showed ketones of > 2 mmol/L already when introducing the MAD. However, blood levels did not correlate with the improvement of symptoms. No significant side effects were observed.

Conclusion: The retrospective analysis of our small group of patients with GLUT1DS demonstrates that MAD as a good and feasible alternative treatment compared with conventional KD.

Keywords: GLUT1DS, modified Atkins diet.