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Cortical tissue of patients with hereditary cerebral hemorrhage with amyloidosis (Dutch) contains various extracellular matrix deposits.
[hereditary cerebral hemorrhage with amyloidosis]
Hereditary
cerebral
hemorrhage
with
amyloidosis
(
Dutch
)
(
HCHWA-D
)
and
Alzheimer
's
disease
(
AD
)
have
certain
clinical
and
histopathologic
features
in
common
;
both
are
characterized
by
cerebral
amyloid
beta
deposits
.
Extracellular
matrix
(
ECM
)
components
have
been
demonstrated
in
plaques
and
in
vascular
amyloid
of
AD
,
suggesting
a
causative
relation
.
We
studied
the
immunohistochemical
expression
of
the
ECM
components
collagen
type
I
,
III
,
and
IV
,
heparan
sulfate
proteoglycan
(
HSPG
)
,
laminin
,
and
fibronectin
in
cortical
tissue
of
six
HCHWA-D
patients
,
two
AD
patients
,
and
five
controls
.
The
vasculature
of
control
patients
and
the
noncongophilic
vessels
in
HCHWA-D
and
AD
patients
stained
for
all
ECM
components
,
with
the
exception
of
collagen
type
I
in
part
of
the
capillaries
.
The
media
of
normal
larger
vessels
stained
for
collagen
type
I
and
III
and
fibronectin
;
a
similar
pattern
was
found
in
the
vascular
amyloid
in
HCHWA-D
and
AD
patients
.
The
plaques
in
HCHWA-D
and
AD
reacted
for
HSPG
and
weakly
for
collagen
III
and
IV
and
laminin
.
Furthermore
,
with
the
exception
of
anti-collagen
type
I
,
all
anti-
ECM
Ab
decorated
coarse
deposits
clustered
in
the
vicinity
of
thick
-walled
,
amyloid-laden
vessels
in
HCHWA-D
patients
;
these
deposits
were
not
spatially
related
to
amyloid
beta
-
positive
plaques
.
This
study
demonstrates
the
presence
of
ECM
components
in
the
main
pathologic
features
of
HCHWA-D
and
as
perivascular
deposits
.
The
observed
distribution
of
matrix
molecules
is
partly
different
from
AD
.
The
ECM
may
well
be
essential
for
the
evolvement
of
the
pathology
of
HCHWA-D
.
Diseases
Validation
Diseases presenting
"cerebral hemorrhage"
symptom
cohen syndrome
hereditary cerebral hemorrhage with amyloidosis
sneddon syndrome
This symptom has already been validated