Use of modified Atkins diet in glucose transporter type 1 deficiency syndrome

Abstract

MAD, which is a less restrictive and more palatable diet than the ketogenic diet, seems to have comparable effectiveness. Moreover, a switch from the ketogenic diet to MAD appears to be beneficial for patients with GLUT1-DS.

Advances in Ketogenic Diet Therapies in Pediatric Epilepsy

“…Some studies report that the MAD is as effective as the classic KD; although, the classic KD is associated with a higher likelihood of seizure freedom in children under 2 years of age with RE [13,14] and epileptic individuals with myoclonic atonic seizures [13]. In addition to its advantages in regard to growth and physical abilities, the MAD can be a good option for long-term maintenance for Glut1DS patients [15]. Nevertheless, for infants with Glut1DS, the classic KD is still considered superior in the early course of treatment and is recommended for long-term maintenance if possible [1].…”

Individualizing Treatment Approaches for Epileptic Patients with Glucose Transporter Type1 (GLUT-1) Deficiency

“…Moreover, the other diet type including the modified atkins diet (MAD) contains unlimited proteins and provides carbohydrates amounts in lower amounts for children (10 g/day) compared to adults (15 g/day) with a 1:1 fat-to-nonfat ratio and is less restrictive alternative compared to the classic KD in terms of discipline and maintaining the compliance. However, using the MAD in the GLUT1 DS patients were shown to have comparable efficacy with KD with a lower ketosis, improved taste, easier daily management, and better selection of dietary foods [ 7 , 8 , 9 ]. Differences exist also in the indications of this diet corresponding to clinical phenotypes and age groups as follows: the classic ketogenic diet 4:1 carbohydrate is indicated in severe phenotypes in infants; the classic ketogenic diet 3:1 carbohydrate is indicated more in mild phenotypes in infants, both phenotypes in pre-school age, school age, adolescent, adults; and MAD is indicated in mild phenotypes in school age, adolescents, and adults [ 9 , 10 ].…”

Alternative ketogenic diets in glucose transporter type 1 deficiency syndrome

“…0.25mM serum β –hydroxybutyrate) seem to be enough to control neurological symptoms, but the relationship between ketosis and long‐term intellectual development is still unknown . Ketone body levels were not reported by Amalou et al…”

“…Amalou et al report 10 patients with GLUT1‐DS treated with MAD for 2.5 years. The outcomes they observed are similar, even in infants, to reports of treatment with the classical ketogenic diet.…”

“…Unfortunately, the delay in diagnosing GLUT1‐DS continues in clinical practice . Amalou et al reported a mean age at diagnosis of 6.2 years (standard deviation [SD] 1.7), whereas the first signs were noted at the age of 1.5 years (SD 0.4).…”