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Emotional disturbance in CADASIL: its impact on quality of life and caregiver burden.
[cadasil]
Recurrent
strokes
and
cognitive
dysfunction
are
the
major
symptoms
of
cerebral
autosomal
dominant
arteriopathy
with
subcortical
infarcts
and
leukoencephalopathy
(
CADASIL
)
.
However
,
emotional
disturbances
in
CADASIL
patients
are
incompletely
understood
.
The
aim
of
this
study
was
to
investigate
emotional
disturbances
in
CADASIL
and
their
impact
on
the
patients
'
quality
of
life
(
QOL
)
and
caregiver
burden
.
From
54
patients
who
were
diagnosed
as
CADASIL
between
January
2000
and
August
2012
in
the
Asan
Medical
Center
,
Seoul
,
Korea
,
23
patients
were
enrolled
in
this
study
.
The
Montgomery
-
Asberg
Depression
Rating
Scale
was
used
for
the
assessment
of
depressive
emotional
disturbances
(
DED
)
.
For
nondepressive
emotional
disturbances
(
NDED
)
,
the
criteria
of
Kim
and
Choi-
Kwon
[
Neurology
2000
;
54
:
1805
-
1810
]
were
used
for
emotional
incontinence
(
excessive
/
inappropriate
expression
of
laughing
or
crying
)
,
and
the
modified
Spielberger
Trait
Anger
Scale
was
used
for
anger
proneness
(
excessive
/
inappropriate
expression
of
anger
)
.
Patients
'
QOL
and
caregiver
burden
were
assessed
with
stroke
-
specific
emotional
QOL
and
the
Sense
of
Competence
Questionnaire
(
SCQ
)
,
respectively
.
Functional
disability
was
assessed
by
the
modified
Rankin
scale
(
mRS
)
,
and
white
matter
ischemic
changes
and
microbleeds
were
analyzed
using
brain
magnetic
resonance
images
.
Twelve
patients
(
52
.
2
%
)
had
various
emotional
disturbances
including
DED
(
n
=
10
,
43
.
5
%
)
and
NDED
(
n
=
7
,
30
.
4
%
)
.
The
presence
of
any
emotional
disturbances
was
associated
with
thalamic
(
p
=
0
.
012
)
and
cortical
(
p
=
0
.
037
)
microbleeds
,
mRS
(
p
=
0
.
001
)
,
cognitive
impairment
(
p
=
0
.
002
)
,
patients
'
low
QOL
(
p
=
0
.
009
)
and
increased
caregiver
burden
(
p
=
0
.
002
)
.
DED
was
associated
with
multiple
(
≥
10
)
microbleeds
(
p
=
0
.
039
)
,
cognitive
impairment
(
p
=
0
.
030
)
and
mRS
(
p
=
0
.
030
)
,
and
negatively
influenced
all
domains
of
patients
'
QOL
and
caregiver
burden
.
NDED
was
associated
with
cortical
microbleeds
(
p
=
0
.
017
)
and
mRS
(
p
=
0
.
014
)
.
Unlike
DED
,
NDED
was
not
associated
with
patients
'
poor
QOL
,
except
for
thinking
domain
,
but
was
significantly
related
to
total
SCQ
and
subscales
1
and
2
of
SCQ
(
p
=
0
.
012
)
.
More
than
half
the
CADASIL
patients
had
emotional
disturbances
,
either
DED
or
NDED
.
Both
are
associated
with
patients
'
poor
QOL
and
increased
caregiver
burden
,
the
former
more
markedly
than
the
latter
.
Considering
that
CADASIL
is
a
progressive
disease
with
deteriorating
patients
'
QOL
,
physicians
have
to
pay
more
attention
to
emotional
problems
in
CADASIL
patients
.
Treatment
strategies
should
be
investigated
in
this
regard
to
improve
patients
'
QOL
and
reduce
caregiver
burden
.
Diseases
Validation
Diseases presenting
"depression"
symptom
22q11.2 deletion syndrome
achondroplasia
adrenal incidentaloma
adrenomyeloneuropathy
alexander disease
aromatase deficiency
cadasil
child syndrome
classical phenylketonuria
congenital adrenal hyperplasia
congenital toxoplasmosis
cushing syndrome
cutaneous mastocytosis
dracunculiasis
fabry disease
familial hypocalciuric hypercalcemia
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
krabbe disease
locked-in syndrome
malignant atrophic papulosis
oligodontia
oral submucous fibrosis
phenylketonuria
sneddon syndrome
triple a syndrome
trochlear dysplasia
von hippel-lindau disease
This symptom has already been validated