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Use of modified Atkins diet in glucose transporter type 1 deficiency syndrome
Author(s) -
Amalou Sofiane,
Gras Domitille,
Ilea Adina,
Greneche MarieOdile,
Francois Laurent,
Bellavoine Vanina,
Delanoe Catherine,
Auvin Stéphane
Publication year - 2016
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/dmcn.13167
Subject(s) - ketogenic diet , glut1 , glucose transporter , medicine , glucose transporter type 1 , epilepsy , pediatrics , ketosis , endocrinology , psychiatry , diabetes mellitus , insulin
Aim Glucose transporter type 1 deficiency syndrome ( GLUT 1‐ DS ) results from impaired glucose transport into the brain, and is treated with a ketogenic diet. A few reports have suggested effectiveness of treatment using the modified Atkins diet ( MAD ). We aimed to assess the efficacy of MAD as a treatment for GLUT 1‐ DS . Method We evaluated the efficacy of MAD in 10 patients (four males, six females; mean age at diagnosis [ SD ] 6.2y [1.7], min–max: 4mo–12y) with GLUT 1‐ DS . Results MAD was started at diagnosis in eight patients, including two infants. The mean duration ( SD ) under MAD was 2.5 [0.6] years (range 6mo–6y). Seven patients with epilepsy started MAD at GLUT 1‐ DS diagnosis, and all experienced improvements in their epilepsy: five out of seven were seizure‐free at M1, and three out of six at M3 and M6. The initiation of MAD allowed symptoms to be controlled in the three patients with movement disorders but without seizures. Two patients switched from the ketogenic diet to MAD . This switch was not responsible for the recurrence of any symptoms, and led to improvements in both physical abilities and growth parameters. Interpretation 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 GLUT 1‐ DS .