Uncommon use of intermittent glucose administration for infrequent non-epileptic paroxysmal events in GLUT1-DS.

ABSTRACT

The ketogenic diet is the treatment of GLUT1 deficiency syndrome that provides an alternative energy source for the brain. However, there are some limitations, including compliance issues as well as patients who do not respond to the ketogenic diet. We report the case of two patients that were not on any particular diet. Both experienced infrequent paroxysmal non-epileptic events (acute ataxia and exercise-induced dystonia). Intermittent glucose intake prior to physical activity for exercise-induced symptoms and at the onset of symptoms for acute ataxia showed consistent and reproducible improvement of the symptoms. Our observations raised the question of developing a new treatment strategy with the induction of a sustained increase in blood glucose. For now, the use of this strategy should be limited to a small group of GLUT1-DS patients who are not on a ketogenic diet.