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Secondary microvascular degeneration in amyloid angiopathy of patients with hereditary cerebral hemorrhage with amyloidosis, Dutch type (HCHWA-D).
[hereditary cerebral hemorrhage with amyloidosis]
Various
secondary
microvascular
degenerative
and
inflammatory
alterations
may
complicate
cerebral
amyloid
angiopathy
(
CAA
)
and
contribute
to
the
morbidity
of
CAA-associated
stroke
.
We
have
investigated
the
severity
of
CAA-associated
microangiopathy
in
a
genetically
determined
Dutch
form
of
CAA
(
HCHWA-D
)
that
has
major
similarities
to
the
type
of
CAA
that
more
commonly
occurs
with
aging
or
Alzheimer
's
disease
(
AD
)
.
The
presence
and
extent
of
the
following
vascular
abnormalities
was
assessed
:
(
1
)
hyalinization
/
fibrosis
,
(
2
)
microaneurysm
formation
,
(
3
)
chronic
(
especially
lymphocytic
)
inflammation
,
(
4
)
perivascular
multinucleated
giant
cells
/
granulomatous
angiitis
,
(
5
)
macrophages
/
histiocytes
within
the
vessel
wall
,
(
6
)
vessel
wall
calcification
,
(
7
)
fibrinoid
necrosis
,
and
(
8
)
mural
or
occlusive
thrombi
.
(
Of
these
,
calcification
of
CAA-affected
vessel
walls
has
,
to
our
knowledge
,
been
described
in
only
a
single
patient
with
CAA-associated
cerebral
hemorrhage
.
)
Some
of
the
changes
,
such
as
histiocytes
in
blood
vessel
walls
and
the
relationship
of
vascular
hyalinosis
to
amyloid
beta
/
A
4
protein
deposition
,
were
highlighted
by
immunohistochemistry
.
By
assessing
the
numbers
of
sections
in
which
the
changes
were
present
for
each
case
,
a
'
score
'
reflective
of
CAA-associated
angiopathy
could
be
obtained
.
This
'
score
'
was
reproducible
among
several
observers
.
We
suggest
that
it
might
also
be
applicable
to
quantifying
severe
CAA
and
related
microvascular
degenerative
changes
in
patients
with
AD
.
beta
/
A
4
immunoreactivity
was
often
sparse
and
adventitial
(
or
almost
absent
)
in
severely
hyalinized
arterioles
and
microaneurysms
.
However
,
macrophages
were
prominent
in
the
walls
of
such
vessels
and
may
play
a
role
in
the
pathogenesis
and
progression
of
CAA-related
microvasculopathy
.
Diseases
Validation
Diseases presenting
"determined dutch form"
symptom
hereditary cerebral hemorrhage with amyloidosis
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