ESPE Abstracts

Purpose: Modified ketogenic diet (MKD) is one treatment option for intractable epilepsy and metabolic conditions such as glucose transporter type 1 deficiency syndrome (GLUT1-DS) and pyruvate dehydrogenase complex (PDC) deficiency. MKD is a less restrictive diet than the classical ketogenic diet (KD) and thus more tolerable. Childhood is an important period for bone acquisition. Some studies indicate a negative effect on bone mass during KD treatment, probably as a consequence of the chronic acidic environment. Long-term data is missing regarding the effects of MKD on bone mass in children.

Aim: To prospectively assess the effect of MKD on bone mass in children treated with MKD for 24 months.

Methods: The included 23 patients (median age 4.8 years; 12 girls, 11 boys) were evaluated with whole body dual-energy X-ray absorptiometry (DXA) and/or calcaneal DXA and laser (DXL) at baseline and after 12 and 24 months on MKD. Underlying etiologies were genetic epilepsy (n=3), GLUT1-DS (n=6), PDC deficiency (n=5), cortical malformation (n=1), mitochondriopathy (n=1), tuberous sclerosis complex (n=2), encephalitis (n=1), Aicardi syndrome (n=1) and of unknown etiology (n=3). Growth parameters were assessed at baseline, 6, 12 and 24 months. DXA and DXL scans were performed at baseline, 12 and 24 months.

Results: In patients with seizures, 76% responded to the diet with >50% seizure reduction. DXA scans are missing in 11 patients due to low age (<5 years) and movement artefacts. Median (min-max) total body bone mineral density head excluded (TB BMD HE) Z-score was −0.6 (−2.5 to 1.4) at baseline and −0.5 (−2.9 to 1.0) after 24 months, P=0.25. Lumbar spine (LS) BMD Z-score was median −0.7 (−2.2 to 2.0) and −1.05 (−2.5 to 0.6), P=0.41. TB BMD HE Z-score was <−1 in 2 patients at baseline and <−1 in 3 patients after 24 months. LS BMD Z-score was <−1 in 3 patients at baseline and in 4 patients after 24 months. MKD treatment for 24 months did not have an effect on LS and TB bone mineral content (BMC). No differences in fat mass or lean mass were observed during the study period. Calcaneal BMD and BMC increased slightly during the study period, P=0.047 and 0.014, respectively.

Conclusions: This study demonstrates that MKD is effective for seizure reduction. Bone mass remained stable during MKD treatment for 24 months. MKD could be considered an effective and safe treatment option in childhood and adolescence.