Evaluation of the Effect of Parenting Style and Parental Mealtime Actions on the Eating Behavior of Children with Epilepsy.

BACKGROUND: Research on the interaction of parenting style, parents' mealtime behaviors, and children's eating behavior in the presence of chronic disease is limited. This study aimed to investigate the impact of parenting style and parental mealtime actions on the eating behavior of children with epilepsy. METHODS: Thirty-one children with epilepsy, thirty-one healthy children (aged 4-9 years), and their parents were included. The Multidimensional Assessment of Parenting Scale (MAPS), Parent Mealtime Action Scale, Children's Eating Behavior Questionnaire, and Healthy Eating Index (HEI)-2015 were applied. The MAPS, HEI-2015 scores, and body mass index for age Z scores were similar in both groups (p > 0.05). In the epilepsy group, the food approach behavior score was higher, and positive correlations were noted between broadband negative parenting and food approach behavior, and the HEI-2015 score and broadband positive parenting (p < 0.05). Regression analysis showed that broadband negative parenting and snack modeling increased the food approach behavior in the epilepsy group. Owing to the chronic disease, the effects of parent-child interaction on the child's eating behavior in the epilepsy group differed from those of healthy children reported in the literature.

DHDDS-related disease; biallelic missense novel variant causing major severity with an early-onset epilepsy and hyperkinetic movement disorder.

BACKGROUND: Dehydrodolichyl diphosphate synthase complex is encoded by DHDDS. De novo mutations in this gene are associated with epilepsy, movement disorders, intellectual and motor disabilities. The clinical picture is commonly identified in children and shows variations in terms of age of onset, severity, seizure types, and types of dyskinesia. CASE: we present a case with a infantile- onset epilepsy and severe global developmental delay, caused by a novel, de novo homozygous variant (c.425C > T, p.Thr142Met) in DHDDS. Clinical improvement was achieved with valproate and tetrabenazine treatments in the 2-year-old male patient with drug-resistant epilepsy, hyperkinetic movement disorder and myoclonus. CONCLUSION: Despite being rare, DHDDS-related diseases should be considered in patients with movement disorders, seizures and global developmental delay in infancy in differential diagnosis of patients resembling neuronal ceroid lipofuscinosis or progressive myoclonic epilepsies.