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Developmental medicine and child neurology v.58 no.11, 2016년, pp.1193 - 1199   SCI SCIE
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Use of modified Atkins diet in glucose transporter type 1 deficiency syndrome

Amalou, Sofiane (Service de Neurologie Pédiatrique, AP‐HP, Hôpital Robert Debré, Paris, France<country scheme="USMARC"> ); Gras, Domitille (fr</country> ); Ilea, Adina (Service de Neurologie Pédiatrique, AP‐HP, Hôpital Robert Debré, Paris, France<country scheme="USMARC"> ); Greneche, Marie‐Odile (fr</country> ); Francois, Laurent (Service de Neurologie Pédiatrique, AP‐HP, Hôpital Robert Debré, Paris, France<country scheme="USMARC"> ); Bellavoine, Vanina (fr</country> ); Delanoe, Catherine (Service de Neurologie Pédiatrique, AP‐HP, Hôpital Robert Debré, Paris, France<country scheme="USMARC"> ); Auvin, Stéphane (fr</country> );
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    Aim Glucose transporter type 1 deficiency syndrome (GLUT1‐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 GLUT1‐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 GLUT1‐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 GLUT1‐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 GLUT1‐DS.


    What this paper adds All patients with seizures (glucose transporter type 1 deficiency syndrome) showed improvement with the modified Atkins diet.Movement disorders and other neurological symptoms were improved by the modified Atkins diet. This article is commented on by De Laet on pages 1106–1107 of this issue.


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