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Influence of vascular risk factors and neuropsychological profile on functional performances in CADASIL: results from the MIcrovascular LEukoencephalopathy Study (MILES).
[cadasil]
Cerebral
autosomal
dominant
arteriopathy
with
subcortical
infarcts
and
leukoencephalopathy
(
CADASIL
)
is
an
inherited
cerebral
small
vessel
disease
that
may
lead
to
disability
and
whose
phenotype
modulators
are
still
unknown
.
In
the
MIcrovascular
LEukoencephalopathy
Study
(
MILES
)
,
we
assessed
the
influence
of
vascular
risk
factors
and
the
effect
of
different
cognitive
domains
(
memory
,
psychomotor
speed
and
executive
functions
)
performances
on
functional
abilities
in
CADASIL
in
comparison
with
age-related
leukoencephalopathy
(
ARL
)
.
We
evaluated
51
CADASIL
patients
(
mean
age
50
.
3
±
13
.
8
years
,
47
.
1
%
males
)
and
68
ARL
patients
(
70
.
6
±
7
.
4
years
,
58
.
8
%
males
)
.
Considering
vascular
risk
factors
,
after
adjustment
for
age
,
CADASIL
patients
had
higher
mean
BMI
values
than
ARL
patients
.
Stroke
history
frequency
was
similar
in
the
two
groups
.
After
adjustment
for
age
,
more
CADASIL
patients
were
disabled
(
impaired
on
≥
2
items
of
the
Instrumental
Activities
of
Daily
Living
scale
)
in
comparison
with
ARL
patients
,
and
CADASIL
patients
had
worse
functional
performances
evaluated
with
the
Disability
Assessment
for
Dementia
(
DAD
)
scale
.
In
CADASIL
patients
,
hypertension
was
related
to
both
DAD
score
and
disability
.
The
cognitive
profile
of
CADASIL
and
ARL
patients
was
similar
,
but
on
a
stepwise
linear
regression
analysis
functional
performances
were
mainly
associated
with
the
memory
index
(
β
=
-
0
.
418
,
P
<
0
.
003
)
in
CADASIL
patients
and
the
executive
function
index
(
β
=
-
0
.
321
,
P
=
0
.
028
)
in
ARL
.
T
his
study
suggests
that
hypertension
may
contribute
to
functional
impairment
in
CADASIL
and
that
memory
impairment
has
a
large
influence
on
functional
decline
in
contrast
with
that
observed
in
a
sample
of
subjects
with
ARL
.
Diseases
Validation
Diseases presenting
"large influence on functional decline"
symptom
cadasil
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