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Microvasculopathy is associated with the number of cerebrovascular lesions in hereditary cerebral hemorrhage with amyloidosis, Dutch type.
[hereditary cerebral hemorrhage with amyloidosis]
Microvascular
changes
such
as
microaneurysms
and
fibrinoid
necrosis
have
been
found
in
the
presence
of
cerebral
amyloid
angiopathy
(
CAA
)
.
These
CAA-associated
microvasculopathies
(
CAA-
AM
)
may
contribute
to
the
development
of
CAA-associated
hemorrhages
and
/
or
infarcts
,
hereafter
referred
to
as
"
cerebrovascular
lesions
.
"
Hereditary
cerebral
hemorrhage
with
amyloidosis
,
Dutch
type
(
HCHWA-D
)
is
an
autosomal
dominant
form
of
CAA
,
in
which
the
amyloid
angiopathy
is
pathologically
and
biochemically
similar
to
sporadic
CAA
associated
with
aging
and
Alzheimer
disease
.
To
determine
the
significance
of
CAA-
AM
for
CAA-associated
cerebrovascular
complications
,
we
investigated
the
association
between
CAA-
AM
and
cerebrovascular
lesions
in
HCHWA-D
patients
.
In
a
previous
autopsy
study
we
semiquantitatively
scored
CAA-
AM
in
29
HCHWA-D
patients
.
In
the
present
study
we
reviewed
clinical
charts
and
autopsy
protocols
of
these
same
patients
.
We
investigated
whether
CAA-
AM
was
associated
with
age
at
death
,
number
of
cerebrovascular
lesions
,
duration
of
clinical
illness
,
hypertension
,
and
atherosclerosis
.
An
association
was
found
between
CAA-
AM
and
the
number
of
cerebrovascular
lesions
(
P
=
0
.
009
)
.
The
presence
of
microaneurysmal
degeneration
was
most
strongly
associated
with
the
number
of
cerebrovascular
lesions
(
P
<
0
.
001
)
.
In
addition
,
we
found
an
association
between
atherosclerosis
and
the
CAA-
AM
score
(
P
=
0
.
047
)
.
Hypertension
was
not
associated
with
CAA-
AM
.
Our
findings
support
previous
reports
suggesting
an
important
role
of
secondary
microvascular
degenerative
changes
in
CAA-associated
cerebrovascular
lesions
and
suggest
an
aggravating
effect
of
systemic
atherosclerosis
,
but
not
hypertension
,
on
the
evolution
of
CAA-
AM
.
These
findings
may
be
of
relevance
to
understanding
cerebrovascular
complications
of
sporadic
or
Alzheimer
disease
-associated
CAA
.
Diseases
Validation
Diseases presenting
"aging"
symptom
cadasil
hereditary cerebral hemorrhage with amyloidosis
inclusion body myositis
severe combined immunodeficiency
werner syndrome
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