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

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.

Anatomic study of juxta renal aneurysms: impact on fenestrated stent-grafts.

BACKGROUND: Fenestrated stent-grafts allow for treatment of patients with juxtarenal aneurysms (JRA) when they present with contraindications for conventional treatment. The fenestrated module is a custom-made module, specially designed to fit a specific patient, using computed tomographic scan measurements, which entails manufacturing delay and high cost. The aim of our study was to evaluate the possibility to reproduce the interrenal aorta anatomy to design a standard fenestrated module that would fit the maximum number of patients with JRA. METHODS: On a three-dimensional working station, we analyzed 289 preoperative computed tomographic scan results of patients with JRA and who were treated with fenestrated stent-grafts comprising two fenestrations for the renal arteries and a scallop for the superior mesenteric artery (SMA). On curvilinear reconstructions, we successively measured the interrenal aorta diameter, its orientation, as well as the height of each renal ostium, taking the ostium center of the SMA as a reference mark. Later, a statistical analysis of these measures distribution was performed so as to design a fenestrated module that would fit the maximum number of patients. RESULTS: The center of the left renal artery presented with a median orientation of 82.5° (range, 37.5-150) and a median distance of 9 mm (range, 0-30), in relation to the SMA ostium. The ostium center of the right renal artery presented with a median orientation of 285° (range, 240-337.5) and a median distance of 8 mm (range, 3-30), in relation to the SMA ostium. By positioning the current renal fenestrations (6-mm wide), on the basis of the calculated median positions, in our series, only 20% of the patients could be treated with a standard fenestrated module. Should the diameter of these fenestrations be increased by 10 mm, it would then be possible to treat 50% of our patients. CONCLUSION: The anatomy of the interrenal aorta and its branches is quite reproducible to design standard fenestrated stent-grafts that could treat half of the patients with JRA.