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Shyness discriminates between children with 22q11.2 deletion syndrome and Williams syndrome and predicts emergence of psychosis in 22q11.2 deletion syndrome.
[22q11.2 deletion syndrome]
22
q
11
.
2
deletion
syndrome
(
22
q
11
.
2
DS
)
is
a
common
neurogenetic
syndrome
associated
with
high
rates
of
psychosis
.
The
aims
of
the
present
study
were
to
identify
the
unique
temperament
traits
that
characterize
children
with
22
q
11
.
2
DS
compared
to
children
with
Williams
syndrome
(
WS
)
and
typically
developing
(
TD
)
controls
,
and
to
examine
temperamental
predictors
of
the
emergence
of
psychosis
in
22
q
11
.
2
DS
.
T
he
temperament
of
55
children
with
22
q
11
.
2
DS
,
36
with
WS
,
and
280
TD
children
was
assessed
using
the
Emotionality
,
Activity
,
Sociability
(
EAS
)
Temperament
Survey
,
Parental
Ratings
.
The
presence
of
a
psychotic
disorder
was
evaluated
in
49
children
and
adolescents
with
22
q
11
.
2
DS
at
baseline
and
again
5
.
43
 
±
 
2
.
23
Â
years
after
baseline
temperament
assessment
.
Children
with
22
q
11
.
2
DS
scored
higher
on
Shyness
compared
to
WS
and
TD
controls
.
Children
with
22
q
11
.
2
DS
and
WS
scored
higher
on
Emotionality
and
lower
on
Activity
compared
to
TD
controls
.
Shyness
was
more
severe
in
older
compared
to
younger
children
with
22
q
11
.
2
DS
.
Baseline
Shyness
scores
significantly
predicted
the
later
emergence
of
a
psychotic
disorder
at
follow-up
,
in
children
with
22
q
11
.
2
DS
.
O
ur
results
suggest
that
shyness
is
an
early
marker
associated
with
the
later
emergence
of
psychosis
in
22
q
11
.
2
DS
.
Diseases
Validation
Diseases presenting
"shyness"
symptom
22q11.2 deletion syndrome
This symptom has already been validated