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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
"blood vessels"
symptom
cadasil
coats disease
cushing syndrome
esophageal adenocarcinoma
hereditary cerebral hemorrhage with amyloidosis
hydrocephalus with stenosis of the aqueduct of sylvius
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
oral submucous fibrosis
pyomyositis
sneddon syndrome
von hippel-lindau disease
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