Lower pre-pulse inhibition in children with the 22q11 deletion syndrome

Christina Sobin, University of Texas, El Paso
Karen Kiley-Brabeck
Maria Karayiorgou

Abstract

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.

Method: 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.