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Microvascular pathology and morphometrics of sporadic and hereditary small vessel diseases of the brain.
[cadasil]
Small
vessel
diseases
(
SVDs
)
of
the
brain
are
likely
to
become
increasingly
common
in
tandem
with
the
rise
in
the
aging
population
.
In
recent
years
,
neuroimaging
and
pathological
studies
have
informed
on
the
pathogenesis
of
sporadic
SVD
and
several
single
gene
(
monogenic
)
disorders
predisposing
to
subcortical
strokes
and
diffuse
white
matter
disease
.
However
,
one
of
the
limitations
toward
studying
SVD
lies
in
the
lack
of
consistent
assessment
criteria
and
lesion
burden
for
both
clinical
and
pathological
measures
.
Arteriolosclerosis
and
diffuse
white
matter
changes
are
the
hallmark
features
of
both
sporadic
and
hereditary
SVDs
.
The
pathogenesis
of
the
arteriopathy
is
the
key
to
understanding
the
differential
progression
of
disease
in
various
SVDs
.
Remarkably
,
quantification
of
microvascular
abnormalities
in
sporadic
and
hereditary
SVDs
has
shown
that
qualitatively
the
processes
involved
in
arteriolar
degeneration
are
largely
similar
in
sporadic
SVD
compared
with
hereditary
disorders
such
as
cerebral
autosomal
arteriopathy
with
subcortical
infarcts
and
leukoencephalopathy
(
CADASIL
)
.
Important
significant
regional
differences
in
lesion
location
within
the
brain
may
enable
one
to
distinguish
SVDs
,
where
frontal
lobe
involvement
appears
consistently
with
almost
every
SVD
,
but
others
bear
specific
pathologies
in
other
lobes
,
such
as
the
temporal
pole
in
CADASIL
and
the
pons
in
pontine
autosomal
dominant
microangiopathy
and
leukoencephalopathy
or
PADMAL
.
Additionally
,
degenerative
changes
in
the
vascular
smooth
muscle
cells
,
the
cerebral
endothelium
and
the
basal
lamina
are
often
rapid
and
more
aggressive
in
genetic
disorders
.
Further
quantification
of
other
microvascular
elements
and
even
neuronal
cells
is
needed
to
fully
characterize
SVD
pathogenesis
and
to
differentiate
the
usefulness
of
vascular
interventions
and
treatments
on
the
resulting
pathology
.
Diseases
Validation
Diseases presenting
"small vessel diseases"
symptom
cadasil
sneddon syndrome
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