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Hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D): II--A review of histopathological aspects.
[hereditary cerebral hemorrhage with amyloidosis]
Cerebral
amyloid-
beta
(
A
beta
)
angiopathy
is
the
histopathological
hallmark
of
hereditary
cerebral
hemorrhage
with
amyloidosis
(
Dutch
)
(
HCHWA-D
)
.
A
beta
deposits
are
found
mainly
in
the
cerebral
and
cerebellar
meningocortical
blood
vessels
and
as
plaques
throughout
the
cerebrocortical
gray
matter
.
A
beta
deposition
in
arteries
and
arterioles
starts
at
the
junction
of
media
and
adventitia
and
proceeds
to
involve
the
media
causing
degeneration
of
the
vascular
smooth
muscle
cells
.
Cerebrocortical
arterioles
often
show
one
or
two
layers
of
radial
A
beta
around
a
layer
of
homogenous
A
beta
that
replaces
the
media
.
Degenerating
neurites
,
reactive
astrocytes
and
microglial
cells
may
surround
cerebrocortical
angiopathic
arterioles
and
capillaries
,
probably
in
reaction
to
invasion
of
the
perivascular
neuropil
by
A
beta
fibrils
.
Furthermore
,
clusters
of
coarse
extracellular
matrix
deposits
may
be
found
near
A
beta
-laden
cerebrocortical
arterioles
.
The
amyloid-associated
proteins
,
cystatin
C
,
and
beta
PP
colocalize
diffusely
with
Dutch
vascular
A
beta
,
whereas
HLA-
DR
immunoreactivity
is
found
only
in
the
periphery
of
the
diseased
vessel
wall
.
The
latter
phenomenon
may
be
related
to
the
presence
of
perivascular
cells
.
Angiopathic
blood
vessels
frequently
show
structural
changes
.
The
relation
of
the
described
pathology
to
the
development
of
hemorrhage
,
infarction
and
leukoencephalopathy
needs
further
elucidation
.
Diseases
Validation
Diseases presenting
"amyloidosis"
symptom
cadasil
fabry disease
familial mediterranean fever
hereditary cerebral hemorrhage with amyloidosis
lymphangioleiomyomatosis
phenylketonuria
systemic capillary leak syndrome
triple a syndrome
waldenström macroglobulinemia
This symptom has already been validated