Lower prepulse inhibition in children with the 22q11 deletion syndrome.
OBJECTIVE
The 22q11 deletion syndrome is associated with a range of possible physical anomalies, probable ongoing learning disabilities, and a specific constellation of neuropsychological deficits, including impairments in selective and executive visual attention, working memory, and sensorimotor functioning. It has been estimated that 25% of the children with 22q11 deletion syndrome go on to develop schizophrenia in late adolescence or adulthood. This is of urgent concern. Specification of early brain network vulnerabilities may provide a basis for early intervention while indicating critical links between genes and severe psychiatric illness. Neuropsychological studies of children with 22q11 deletion syndrome have implicated an array of potentially aberrant brain pathways. This study was conducted to determine whether preattentive processing ("sensorimotor gating") deficits are present in this population.


METHODS
The authors administered a test of prepulse inhibition to 25 children with 22q11 deletion syndrome and their 23 sibling comparison subjects, ages 6-13. It was predicted that the children with 22q11 deletion syndrome would have lower prepulse inhibition than the comparison subjects.


RESULTS
Prepulse inhibition in the children with 22q11 deletion syndrome (26.06%) was significantly less than that of the sibling comparison subjects (46.41%). Secondary analyses suggested that this decrement did not reflect developmental delay, and lower prepulse inhibition was associated with particular subsyndromal symptoms in some children.


CONCLUSIONS
Sensorimotor gating is lower in children with 22q11 deletion syndrome. These findings may indicate specific brain circuits that are anomalous in 22q11 deletion syndrome.
Sobin
Christina
C
Laboratory of Human Neurogenetics, Rockefeller University, New York, NY 10021, USA. sobinc@rockefeller.edu
Kiley-Brabeck
Karen
K
Karayiorgou
Maria
M
D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000235:genetics; D000161:Acoustic Stimulation; D000293:Adolescent; D000328:Adult; D001767:Blinking / Q000502:physiology; D002648:Child; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics*; D019465:Craniofacial Abnormalities / Q000175:diagnosis / Q000235:genetics; D002658:Developmental Disabilities / Q000175:diagnosis / Q000235:genetics; D004062:DiGeorge Syndrome / Q000175:diagnosis / Q000235:genetics; D005260:Female; D017353:Gene Deletion; D006801:Humans; D007859:Learning Disabilities / Q000175:diagnosis / Q000235:genetics; D008297:Male; D011569:Psychiatric Status Rating Scales; D011930:Reaction Time / Q000235:genetics / Q000502:physiology; D013216:Reflex, Startle / Q000235:genetics / Q000502:physiology*; D035781:Siblings / Q000523:psychology; D013577:Syndrome