The Role of Carnitine Monitoring and Supplementation in Children with Epilepsy on a Ketogenic Diet
Abstract number :
2.003
Submission category :
10. Dietary Therapies (Ketogenic, Atkins, etc.)
Year :
2025
Submission ID :
543
Source :
www.aesnet.org
Presentation date :
12/7/2025 12:00:00 AM
Published date :
Authors :
Presenting Author: Emma Vail, BS – Johns Hopkins University School of Medicine
Eric Kossoff, MD – Johns Hopkins University School of Medicine
Zahava Turner, RD – Johns Hopkins Hospital
Rationale: There has been limited research on carnitine levels, supplementation, and the ketogenic diet (KD).
Methods: Over 8 years, 150 consecutive children treated with the KD at Johns Hopkins Hospital were evaluated and information about carnitine levels and use obtained.
Results: One hundred and five (70%) had carnitine levels checked. The mean total carnitine level at first follow-up was 56 µmol/L (SD 32)(normal range 30-60 µmol/L) and free 26 (SD 19) µmol/L (normal range 22-52 µmol/L). In those not supplemented with carnitine, total carnitine was stable (46.2 (SD 12) to 44.9 (SD 19) µmol/L, p=0.80), whereas free carnitine decreased (35.8 (SD 12) to 20.1 (SD 11) µmol/L, p< 0.001). Those on valproate had lower baseline total carnitine levels (40.7 (SD 20) vs. 52.0 (SD 15) µmol/L, p=0.02). At 3 months, 83% with normal total carnitine levels ( >30 µmol/L) had >50% seizure reduction compared to 60% with hypocarnitinemia (p=0.06). Carnitine was supplemented in 36 (24%), typically in those older, on higher ketogenic ratios, prior to 2020, and with longer KD durations. Twelve (33%) had documented benefit from carnitine supplementation, but there was no group difference in seizure control or ketosis.
Conclusions: In this single-center study, hypocarnitinemia was seen at baseline in those on valproate and decreased free carnitine levels occurred over time. Those with higher total carnitine levels at 3 months were slightly more likely to be improved. Carnitine was supplemented in one-quarter of patients, with 1 of 3 showing modest benefit in ketosis and seizures.
Funding: Johns Hopkins University School of Medicine Dean’s Grant for summer research
Dietary Therapies (Ketogenic, Atkins, etc.)