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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
.
Diseases
Validation
Diseases presenting
"dementia"
symptom
adrenomyeloneuropathy
alexander disease
cadasil
canavan disease
carcinoma of the gallbladder
cushing syndrome
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
inclusion body myositis
phenylketonuria
sneddon syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated