Behavioral and temperamental features of children with Costello syndrome.

Cédric Galéra, Marie-Ange Delrue, Cyril Goizet, Kattalin Etchegoyhen, Emmanuelle Taupiac, Sabine Sigaudy, Benoît Arveiler, Nicole Philip, Manuel Bouvard, Didier Lacombe
Am. J. Med. Genet.. 2006-01-01; 140A(9): 968-974
DOI: 10.1002/ajmg.a.31169

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1. Am J Med Genet A. 2006 May 1;140(9):968-74.

Behavioral and temperamental features of children with Costello syndrome.

Galéra C(1), Delrue MA, Goizet C, Etchegoyhen K, Taupiac E, Sigaudy S, Arveiler
B, Philip N, Bouvard M, Lacombe D.

Author information:
(1)Child Psychiatry Department, Centre Hospitalier Charles-Perrens, Bordeaux2
University, Bordeaux, France.

Costello syndrome (CS) is a rare genetic condition due to germline mutations in
HRAS proto-oncogene and characterized by increased birth weight, postnatal growth
retardation, distinctive facial appearance, typical medical problems (including
feeding problems in the neonatal period), cutaneous anomalies, and developmental
delay. Outgoing personality has often been noted in case reports, but few studies
have focused specifically on the behavioral aspects of CS. A preliminary survey
described irritability in younger patients with improvement between age 2 and 4,
but a standardized psychometric tool was not used. A second study using the Child
Behavior Checklist (CBCL) showed relatively high (albeit subclinical) levels of
internalizing problems. These descriptive investigations lacked a control group.
We describe a comparative survey to evaluate the behavioral and temperamental
features of children with CS. We conducted a cross-sectional assessment using the
CBCL and the Emotionality, Activity, Shyness, Sociability (EAS) temperament
questionnaire to evaluate behavior and temperament in 11 CS children (2 years 5
months to 9 years) comparing them to 33 gender- and age-matched children without
disability. The results suggest that the high levels of internalizing problems
found before age 4 in CS patients might decrease with age. They also point to
possible “hyperemotionality.” Further studies using a larger sample size and
IQ-matched control groups are needed to more accurately characterize individuals
with this rare syndrome.

2006 Wiley-Liss, Inc.

DOI: 10.1002/ajmg.a.31169
PMID: 16575889 [Indexed for MEDLINE]

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