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Familial cerebral amyloid angiopathy related to stroke and dementia.
[hereditary cerebral hemorrhage with amyloidosis]
The
term
cerebral
amyloid
angiopathy
(
CAA
)
refers
to
the
specific
deposition
of
amyloid
fibrils
in
the
walls
of
leptomeningeal
and
cortical
arteries
,
arterioles
and
,
although
less
frequently
in
capillaries
and
veins
.
It
is
commonly
associated
with
Alzheimers
disease
,
Down
's
syndrome
and
normal
aging
,
as
well
as
with
a
variety
of
familial
conditions
related
to
stroke
and
/
or
dementia
:
hereditary
cerebral
hemorrhage
with
amyloidosis
of
Icelandic
type
(
HCHWA
-
I
)
,
various
inherited
disorders
linked
to
Abeta
mutants
(
including
the
Dutch
variant
of
HCHWA
)
,
and
the
recently
described
chromosome
13
familial
dementia
in
British
and
Danish
kindreds
.
This
review
focuses
on
four
different
types
of
hereditary
CAA
,
emphasizing
the
notion
that
CAA
is
not
only
related
to
stroke
but
also
to
neurodegeneration
and
dementia
of
the
Alzheimer
's
type
.