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A random Abstract
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Our Team
[Pathomechanisms and treatment of CADASIL].
[cadasil]
The
pathomechanisms
of
cerebral
autosomal
dominant
arteriopathy
with
subcortical
infarcts
and
leukoencephalopathy
(
CADASIL
)
are
still
under
debate
.
Granular
osmiophilic
material
(
GOM
)
,
which
accumulates
around
the
basement
membrane
,
and
the
extracellular
domain
of
NOTCH
3
(
NECD
)
in
the
vessel
are
key
molecules
that
contributes
to
the
destruction
of
smooth
muscle
cells
in
CADASIL
.
In
addition
,
GOM
and
NECD
may
be
related
to
the
dysfunction
of
cerebral
small
vessels
in
patients
with
CADASIL
.
In
this
review
,
the
role
of
the
accumulation
of
these
abnormal
proteins
in
the
cerebral
small
vessels
,
and
the
pathomechanism
from
white
matter
lesions
,
microbleeds
,
and
lacunar
infarctions
to
vascular
dementia
are
discussed
.
We
diagnosed
63
CADASIL
cases
and
identified
3
features
that
were
common
to
Japanese
cases
.
First
,
the
ages
of
onset
of
clinical
symptoms
other
than
migraine
were
widely
distributed
;
the
age
of
onset
of
symptoms
was
greater
than
60
years
in
more
than
20
%
of
the
cases
.
Second
,
65
%
of
the
Japanese
CADASIL
cases
had
stroke
risk
factors
,
such
as
hypertension
,
hyperlipidemia
,
or
smoking
.
Third
,
in
20
%
of
the
cases
,
there
was
no
family
history
of
stroke
.
Therefore
,
new
diagnostic
criteria
for
Japanese
patients
with
CADASIL
were
proposed
on
the
basis
of
these
clinical
features
in
order
to
avoid
missing
cases
of
CADASIL
.
The
criteria
are
useful
for
screening
candidates
of
CADASIL
,
even
in
cases
with
elderly
onset
,
stroke
risk
factors
,
and
obscure
family
history
.
Diseases
Validation
Diseases presenting
"cerebral small vessels in patients"
symptom
cadasil
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