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Psychopathology and cognition in children with 22q11.2 deletion syndrome.
[22q11.2 deletion syndrome]
Children
with
22
q
11
.
2
deletion
syndrome
(
22
q
11
.
2
DS
)
have
been
reported
to
have
high
rates
of
cognitive
and
psychiatric
problems
.
To
establish
the
nature
and
prevalence
of
psychiatric
disorder
and
neurocognitive
impairment
in
children
with
22
q
11
.
2
DS
and
test
whether
risk
of
psychopathology
is
mediated
by
the
children
's
intellectual
impairment
.
Neurocognition
and
psychopathology
were
assessed
in
80
children
with
22
q
11
.
2
DS
(
mean
age
10
.
2
years
,
s
.
d
.
=
2
.
1
)
and
39
sibling
controls
(
mean
age
10
.
9
years
,
s
.
d
.
=
2
.
0
)
.
More
than
half
(
54
%
)
of
children
with
22
q
11
.
2
DS
met
diagnostic
criteria
for
one
or
more
DSM
-IV-TR
psychiatric
disorder
.
These
children
had
lower
IQ
(
mean
76
.
8
,
s
.
d
.
=
13
.
0
)
than
controls
(
mean
108
.
6
,
s
.
d
.
=
15
.
2
)
(
P
<
0
.
001
)
and
showed
a
range
of
neurocognitive
impairments
.
Increased
risk
of
psychopathology
was
not
mediated
by
intellectual
impairment
.
22
q
11
.
2
DS
is
not
related
to
a
specific
psychiatric
phenotype
in
children
.
Moreover
,
the
deletion
has
largely
independent
effects
on
IQ
and
risk
of
psychopathology
,
indicating
that
psychopathology
in
22
q
11
.
2
DS
is
not
a
non-
specific
consequence
of
generalised
cognitive
impairment
.
Diseases
Validation
Diseases presenting
"high rates"
symptom
22q11.2 deletion syndrome
acute rheumatic fever
carcinoma of the gallbladder
congenital diaphragmatic hernia
congenital toxoplasmosis
cowden syndrome
cystinuria
dracunculiasis
dystrophic epidermolysis bullosa
locked-in syndrome
pyomyositis
von hippel-lindau disease
x-linked adrenoleukodystrophy
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