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Cerebral amyloidosis, amyloid angiopathy, and their relationship to stroke and dementia.
[hereditary cerebral hemorrhage with amyloidosis]
Cerebral
amyloid
angiopathy
(
CAA
)
is
the
common
term
used
to
define
the
deposition
of
amyloid
in
the
walls
of
medium-
and
small
-size
leptomeningeal
and
cortical
arteries
,
arterioles
and
,
less
frequently
,
capillaries
and
veins
.
CAA
is
an
important
cause
of
cerebral
hemorrhages
although
it
may
also
lead
to
ischemic
infarction
and
dementia
.
It
is
a
feature
commonly
associated
with
normal
aging
,
Alzheimer
disease
(
AD
)
,
Down
syndrome
(
DS
)
,
and
Sporadic
Cerebral
Amyloid
Angiopathy
.
Familial
conditions
in
which
amyloid
is
chiefly
deposited
as
CAA
include
hereditary
cerebral
hemorrhage
with
amyloidosis
of
Icelandic
type
(
HCHWA
-
I
)
,
familial
CAA
related
to
Abeta
variants
,
including
hereditary
cerebral
hemorrhage
with
amyloidosis
of
Dutch
origin
(
HCHWA-D
)
,
the
transthyretin
-related
meningocerebrovascular
amyloidosis
of
Hungarian
and
Ohio
kindreds
,
the
gelsolin
-related
spinal
and
cerebral
amyloid
angiopathy
,
familial
PrP-CAA
,
and
the
recently
described
chromosome
13
familial
dementia
in
British
and
Danish
kindreds
.
This
review
focuses
on
the
various
molecules
and
genetic
variants
that
target
the
cerebral
vessel
walls
producing
clinical
features
related
to
stroke
and
/
or
dementia
,
and
discusses
the
potential
role
of
amyloid
in
the
mechanism
of
neurodegeneration
.
Diseases
Validation
Diseases presenting
"cerebral hemorrhages"
symptom
cadasil
hereditary cerebral hemorrhage with amyloidosis
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