Neuropsychological, learning and psychosocial profile of primary school aged children with the velo-cardio-facial syndrome (22q11 deletion): evidence for a nonverbal learning disability?
In this exploratory study, the neuropsychological and learning profile of nine primary school age children with velo-cardio-facial syndrome (VCFS) was studied by systematic neuropsychological testing. In five out of nine children, the following profile was found: a VIQ-PIQ discrepancy (in favor of the VIQ), significantly better scores (.05 level) for reading (decoding) and spelling compared to arithmetic, deficient tactile-perceptual skills (difficulties mainly on the left side of the body), weak but not deficient visual-perceptual abilities, deficient visual-spatial skills, extremely poor psychomotor skills (gross motor skills more deficient than fine motor skills), problems with processing of new and complex material, poor visual attention, good auditory memory and relatively good language skills. These findings correspond to the pattern of neuropsychological assets and deficits that has been described for the syndrome of nonverbal learning disabilities (NLD) (Rourke, 1987, 1988, 1989, 1995). The psychosocial profile of all nine children with VCFS also correspond to that of children with NLD. Further studies on the relationship between cognitive function, behavior, psychiatric disorder and abnormalities in brain anatomy in young people with VCFS will be needed. In clinical practice, it is worthwhile exploring in greater depth the neuropsychological functions of children with VCFS to rule out NLD, since they may benefit from specific remediation following the learning principles of the NLD-treatment.
Swillen
A
A
Center for Human Genetics Leuven, University Hospital Gasthuisberg, Belgium. ann.swillen@med.kuleuven.ac.be
Vandeputte
L
L
Cracco
J
J
Maes
B
B
Ghesquière
P
P
Devriendt
K
K
Fryns
J P
JP
D001288:Attention; D002648:Child; D002872:Chromosome Deletion; D002892:Chromosomes, Human, Pair 22; D019465:Craniofacial Abnormalities / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D006801:Humans; D007360:Intelligence / Q000235:genetics; D007859:Learning Disabilities / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D008297:Male; D011939:Mental Recall; D009483:Neuropsychological Tests; D011596:Psychomotor Disorders / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D014681:Velopharyngeal Insufficiency / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D014706:Verbal Learning