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Dementia in hereditary cerebral hemorrhage with amyloidosis-Dutch type is associated with cerebral amyloid angiopathy but is independent of plaques and neurofibrillary tangles.
[hereditary cerebral hemorrhage with amyloidosis]
Cerebral
amyloid
angiopathy
is
frequently
found
in
demented
and
nondemented
elderly
persons
,
but
its
contribution
to
the
causation
of
dementia
is
unknown
.
Therefore
,
we
investigated
the
relation
between
the
amount
of
cerebral
amyloid
angiopathy
and
the
presence
of
dementia
in
19
patients
with
hereditary
cerebral
hemorrhage
with
amyloidosis
-
Dutch
type
.
The
advantage
of
studying
hereditary
cerebral
hemorrhage
in
amyloidosis
-
Dutch
type
is
that
patients
with
this
disease
consistently
have
severe
cerebral
amyloid
angiopathy
with
minimal
neurofibrillary
pathology
.
The
amount
of
cerebral
amyloid
angiopathy
,
as
quantified
by
computerized
morphometry
,
was
strongly
associated
with
the
presence
of
dementia
independent
of
neurofibrillary
pathology
,
plaque
density
,
or
age
.
The
number
of
cortical
amyloid
beta
-laden
severely
stenotic
vessels
,
vessel-within-vessel
configurations
,
and
cerebral
amyloid
angiopathy
-associated
microvasculopathies
was
associated
with
the
amount
of
cerebral
amyloid
angiopathy
and
dementia
.
A
semiquantitative
score
,
based
on
the
number
of
amyloid
beta
-laden
severely
stenotic
vessels
,
completely
separated
demented
from
nondemented
patients
.
These
results
suggest
that
extensive
(
more
than
15
amyloid
beta
-laden
severely
stenotic
vessels
in
five
frontal
cortical
sections
)
cerebral
amyloid
angiopathy
alone
is
sufficient
to
cause
dementia
in
hereditary
cerebral
hemorrhage
with
amyloidosis
-
Dutch
type
.
This
may
have
implications
for
clinicopathological
correlations
in
Alzheimer
's
disease
and
other
dementias
with
cerebral
amyloid
angiopathy
.
Diseases
Validation
Diseases presenting
"completely separated demented from nondemented patients"
symptom
hereditary cerebral hemorrhage with amyloidosis
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