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Objective. The ketogenic diet therapy is a time-tested and potent non-pharmacologic treatment for epilepsy. However, the study of the ketogenic diet in patients with genetic generalized epilepsy (GGE) is not widely established. The aim of this study was to evaluate the efficacy and tolerability of the modified Atkins diet, a variation of the ketogenic diets, as a treatment for drug-resistant GGE.Methods. A retrospective chart review was performed in patients with epilepsy treated with the modified Atkins diet at the University of Chicago from 2017 to 2020. For three months following diet initiation, participants were monitored for diet tolerability and effect on seizures. Response to the treatment was recorded by self-reporting patients and guardians.Results. Thirteen patients with a diagnosis of drug-resistant GGE were identified. An average of 3.8 anti-seizure medications (ASMs) had been tried and 3.4 years had elapsed from seizure onset before dietary therapy was attempted. Patients were receiving a mean of 2.2 ASMs at the time of diet initiation. After undergoing dietary treatment for three months, 12/13 (92%) patients experienced a greater than 50% reduction in seizure frequency, 6/13 (46%) patients became seizure-free, and 7/13 (54%) were able to discontinue at least one ASM. All patients completed at least three months of dietary therapy with an average duration of 9.3 months at the time of report. One patient reported side effects of fatigue which may be attributed to the diet.Significance. The modified Atkins diet has shown to be an effective and well-tolerated treatment for children with drug-resistant GGE. The diet provides the additional benefit of aiding to discontinue ASMs and, therefore, minimize the side effects from polypharmacy. Given these results, it seems reasonable to consider the modified Atkins diet as an alternative and possibly earlier treatment option for patients with drug-resistant GGE.