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<p>We report a&#xa0;retrospective multicenter study on ketogenic diets (KD) administered for at least 3&#xa0;months in 32&#xa0;patients under 18&#xa0;years of age with tuberous sclerosis complex (TSC). The primary endpoint was seizure reduction after 6&#xa0;months under KD. Secondary endpoints were seizure reduction at other time points, tolerability and retention rate. The KD was administered for a&#xa0;median of 9.2&#xa0;months (range 3–132 months). After 6&#xa0;months 27&#xa0;patients were still on the diet. A&#xa0;relevant reduction of seizure frequency was found in 15 (58%) of 26 analyzable cases and 6 became seizure-free (23%). The efficacy of the KD declined over time. At the end of the observation period the KD had been stopped in 27&#xa0;patients. The documented reasons were lack of efficacy (6), noncompliance (8), side effects (5) and surgery&#xa0;(3). Mild and/or transient side effects were documented in 8&#xa0;patients, 5 other patients discontinued KD due to side effects (2 cases of elevated transaminases and 1 case each of pancreatitis, vomiting and kidney stones). Patients in the subgroup with classical KD (<i>N</i> = 19) were significantly younger than those with a&#xa0;modified Atkins diet (<i>N</i> = 13). Efficacy and tolerability did not differ between these subgroups. There was no difference between patients receiving concomitant mTOR inhibition (<i>N</i> = 13) and patients without mTOR inhibition throughout the entire duration of KD (<i>N</i> = 12).</p><p>KD is an effective and well-tolerated epilepsy therapy in TSC. A&#xa0;combination with mTOR inhibitors is possible. The effectiveness of KD in our cohort is comparable to that generally expected for epilepsy; however, we found no evidence that KD was more effective in patients with TSC than in cohorts with other etiologies.</p>

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Ketogene Ernährungstherapie bei Kindern mit tuberöse Sklerose-Komplex

  • 期刊论文

  • 发表日期：

    2026年3月17日

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  • T. Bast,
  • C. Raab,
  • A. Wiemer-Kruel,
  • M. Kämpf,
  • A. de Saint Martin,
  • T. Polster,
  • D. Franz

摘要

We report a retrospective multicenter study on ketogenic diets (KD) administered for at least 3 months in 32 patients under 18 years of age with tuberous sclerosis complex (TSC). The primary endpoint was seizure reduction after 6 months under KD. Secondary endpoints were seizure reduction at other time points, tolerability and retention rate. The KD was administered for a median of 9.2 months (range 3–132 months). After 6 months 27 patients were still on the diet. A relevant reduction of seizure frequency was found in 15 (58%) of 26 analyzable cases and 6 became seizure-free (23%). The efficacy of the KD declined over time. At the end of the observation period the KD had been stopped in 27 patients. The documented reasons were lack of efficacy (6), noncompliance (8), side effects (5) and surgery (3). Mild and/or transient side effects were documented in 8 patients, 5 other patients discontinued KD due to side effects (2 cases of elevated transaminases and 1 case each of pancreatitis, vomiting and kidney stones). Patients in the subgroup with classical KD (N = 19) were significantly younger than those with a modified Atkins diet (N = 13). Efficacy and tolerability did not differ between these subgroups. There was no difference between patients receiving concomitant mTOR inhibition (N = 13) and patients without mTOR inhibition throughout the entire duration of KD (N = 12).

KD is an effective and well-tolerated epilepsy therapy in TSC. A combination with mTOR inhibitors is possible. The effectiveness of KD in our cohort is comparable to that generally expected for epilepsy; however, we found no evidence that KD was more effective in patients with TSC than in cohorts with other etiologies.