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Sporadic and familial cerebral amyloid angiopathies.
[hereditary cerebral hemorrhage with amyloidosis]
Cerebral
amyloid
angiopathy
(
CAA
)
is
the
term
used
to
describe
deposition
of
amyloid
in
the
walls
of
arteries
,
arterioles
and
,
less
often
,
capillaries
and
veins
of
the
central
nervous
system
.
CAAs
are
an
important
cause
of
cerebral
hemorrhage
and
may
also
result
in
ischemic
lesions
and
dementia
.
A
number
of
amyloid
proteins
are
known
to
cause
CAA
.
The
most
common
sporadic
CAA
,
caused
by
A
beta
deposition
,
is
associated
with
aging
and
is
a
common
feature
of
Alzheimer
disease
(
AD
)
.
CAA
occurs
in
several
familial
conditions
,
including
hereditary
cerebral
hemorrhage
with
amyloidosis
of
Icelandic
type
caused
by
deposition
of
mutant
cystatin
C
,
hereditary
cerebral
hemorrhage
with
amyloidosis
Dutch
type
and
familial
AD
with
deposition
of
either
A
beta
variants
or
wild-
type
A
beta
,
the
transthyretin
-related
meningo-
vascular
amyloidoses
,
gelsolin
as
well
as
familial
prion
disease
-related
CAAs
and
the
recently
described
BRI
2
gene
-related
CAAs
in
familial
British
dementia
and
familial
Danish
dementia
.
This
review
focuses
on
the
morphological
,
biochemical
,
and
genetic
aspects
as
well
as
the
clinical
significance
of
CAAs
with
special
emphasis
on
the
BRI
2
gene
-related
cerebrovascular
amyloidoses
.
We
also
discuss
data
relevant
to
the
pathomechanism
of
the
different
forms
of
CAA
with
an
emphasis
on
the
most
common
A
beta
-related
types
.
Diseases
Validation
Diseases presenting
"may also result in ischemic lesions and dementia"
symptom
hereditary cerebral hemorrhage with amyloidosis
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