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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
"psychosis"
symptom
22q11.2 deletion syndrome
child syndrome
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
sneddon syndrome
This symptom has already been validated
