Head circumferences of patients with Dravet syndrome show growth slowdown

Tommaso Lo Barco; Nicole Chemaly; Theo Teng; Francesca Darra; Rima Nabbout

doi:10.1016/j.yebeh.2020.107157

Head circumferences of patients with Dravet syndrome show growth slowdown

Epilepsy Behav. 2020 Oct:111:107157. doi: 10.1016/j.yebeh.2020.107157. Epub 2020 Jul 20.

Authors

Tommaso Lo Barco¹, Nicole Chemaly², Theo Teng², Francesca Darra³, Rima Nabbout⁴

Affiliations

¹ Reference Centre for Rare Epilepsies, Department of Pediatric Neurology, Necker Enfants Malades Hospital, Paris Descartes University, Paris, France; Child Neuropsychiatry, Department of Surgical Sciences, Dentistry, Gynecology and Pediatrics, University of Verona, Italy.
² Reference Centre for Rare Epilepsies, Department of Pediatric Neurology, Necker Enfants Malades Hospital, Paris Descartes University, Paris, France.
³ Child Neuropsychiatry, Department of Surgical Sciences, Dentistry, Gynecology and Pediatrics, University of Verona, Italy.
⁴ Reference Centre for Rare Epilepsies, Department of Pediatric Neurology, Necker Enfants Malades Hospital, Paris Descartes University, Paris, France; Inserm U1163, Imagine institute, Paris, France. Electronic address: rima.nabbout@aphp.fr.

PMID: 32702652
DOI: 10.1016/j.yebeh.2020.107157

Abstract

The measurement of head circumference (HC) represents a useful and reliable tool to monitor brain growth. Many genetic conditions are associated with an abnormal pattern of head growth, but no specific pattern has been described in Dravet Syndrome (DS). To investigate the head growth trajectories in a pediatric population with DS, a retrospective analysis of medical records of patients with DS was performed in 2 epilepsy centers. Quantitative data were compared with z-score growth curve of standard population, and an independent samples t-test was performed using 6-month ranges. A total of 137 subjects aged less than 18 years were included, with a total of 529 HC values and a mean of 3.9 measures per patient. From birth until 24 months of life, HC values were almost equally distributed around the mean trajectory of the reference population from each side of the curve. This trend line deflects from the mean curve after 24 months showing a head growth slowdown reaching a statistical significance (p < .05) from 48 months for males and 60 for females. Future prospective studies are needed to assess factors that can impact head growth and explore possible phenotype-genotype correlation with HC.

Keywords: Developmental and epileptic encephalopathy; Dravet Syndrome; Head circumference.

Publication types

Multicenter Study
Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Cephalometry / methods
Cephalometry / trends*
Child
Child, Preschool
Epilepsies, Myoclonic / diagnosis*
Epilepsies, Myoclonic / physiopathology*
Female
Head / growth & development*
Humans
Male
Prospective Studies
Retrospective Studies