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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
"dementia"
symptom
adrenomyeloneuropathy
alexander disease
cadasil
canavan disease
carcinoma of the gallbladder
cushing syndrome
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
inclusion body myositis
phenylketonuria
sneddon syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated