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Spectrum of neurodevelopmental disabilities in children with cerebellar malformations

Advances in perinatal care and neuroimaging techniques have increased the
detection of cerebellar malformations (CBMs) in the fetus and young infant. As a
result, this has necessitated a greater understanding of the neurodevelopmental
consequences of CBMs on child development. The aim of this study was to delineate
the impact of CBMs on long-term neurodevelopmental outcomes. METHOD: We conducted
a cross-sectional study and systematically identified children with CBMs born
between December 2000 and December 2006. We then performed follow-up magnetic
resonance imaging studies, neurologic examination, and standardized
neurodevelopmental outcome testing (Mullen Scales of Early Learning, Vineland
Adaptive Behavior Scale, Child Behavior Checklist, Modified Checklist for Autism
in Toddlers, and the Pediatric Quality of Life Inventory). RESULTS: Our sample
comprised 49 children (29 males, 20 females; mean age, 28.4 mo, SD 16.4) with a
CBM. Infants with evidence of acquired fetal or neonatal brain injury,
intracranial birth trauma, inherited metabolic disease, or major pre- or
postnatal cerebral ischemia were excluded. Our findings highlight that children
with CBMs experience a high prevalence of neurologic, developmental, and
functional disabilities including motor, cognitive, language, and
social-behavioral deficits, as well as poor quality of life.
The associated
supratentorial anomalies, chromosomal findings, and malformations affecting the
cerebellar vermis were significant independent predictors of neurodevelopmental
disabilities in young children with CBMs. The associated supratentorial anomalies
and chromosomal findings were also predictive of global developmental delay
(p=0.01), cognitive impairment (p=0.03), gross and fine motor delay (p=0.02 and
p=0.01 respectively), and positive screening for autism spectrum disorder
(p=0.01). Additionally, malformations affecting the cerebellar vermis were
significant independent predictors of expressive language (p=0.04) and gross
motor delays (p=0.02). INTERPRETATION: Developmental surveillance and early
intervention programs should be an integral part of the long-term follow-up of
survivors of CBM.
CI - (c) The Authors. Developmental Medicine & Child Neurology (c) 2011 Mac Keith
Press.

Langue : ANGLAIS

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