Mealtime behaviour disorders in Cornelia de Lange syndrome

Abstract

Rationale and objectives: Cornelia de Lange syndrome (CdLS) is a multisystem congenital syndrome at variable expressivity. Children with CdLS can exhibit a range of “autistic-like” behaviours. Only a few studies have investigated the presence of eating "autistic-like" behaviors in individuals with CdLS. The aim of this study is investigating eating disorders and food selectivity among children affected by CdLS compared to autistic spectrum disorder (ASD) and typical developing (TD) children. Materials and methods: 16 children affected by CDLs (10 M, mean age 5.12±1.98 years) were compared to 35 ASD children (20 M, mean age 4.94±1.72 years) and 77 TD children (41 M, mean age 4.78±2.06 years). All subjects were submitted to Brief Autism Mealtime Behavior Inventory (BAMBI). To confirm the diagnosis, all individuals with CdLS and ASD were subjected to Griffiths Mental Developmental Scales (GMDS) and Autism Diagnostic Observation Schedule (ADOS). According to the DSM-5, all children with an ADOS total score ≥ 12 were included in the ASD group. .P values <0.05 were considered statistically significant. Results: Three study groups were comparable for age (p=0.793) and gender (p=0.774) distribution. No significant differences were found between CdLS and ASD groups in Global developmental quotient (GQ) (p=0.636) and in ADOS total score (p=0.426) In CdLS children BAMBI evaluation showed lower total quote of mealtime behavior disorders (p<0.001), Food Selectivity (p<0.001), Disruptive Mealtime Behaviors (p<0.001), and Mealtime Rigidity (p<0.001) than ASD children. No significant differences were found for Food Refusal (p=0.118) among three groups. Conclusions: CdLS children showed a higher prevalence of eating "autistic-like” disorders. However, profile and developmental paths of these symptoms are different from those observed in ASD childern. The results of this study suggest that eating behavioral abnormalities at the time of the meal, a symptom commonly observed in ASD, are usually not part of the behavioral symptoms accompanying children with CdLS.