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Clinical aspects of cerebral amyloid angiopathy.
[hereditary cerebral hemorrhage with amyloidosis]
Amyloid
depositions
mainly
consist
of
proteins
with
a
fibrillary
structure
.
A
large
number
of
different
proteins
have
amyloidogenic
properties
.
Amyloids
are
now
categorized
on
the
basis
of
their
chemical
structure
,
but
the
clinical
classification
of
localized
and
systemic
amyloid
is
still
useful
.
The
proteins
that
can
be
found
in
cerebral
amyloid
angiopathy
are
cystatin
C
,
beta
/
A
4
and
transthyretin
.
Cerebral
amyloid
angiopathy
can
remain
symptom-free
,
but
can
also
give
a
broad
spectrum
of
clinical
and
radiological
manifestations
,
including
(
vascular
)
dementia
,
cerebellar
and
cerebral
hemorrhage
,
subarachnoid
hemorrhage
,
and
leukoencephalopathy
.
It
is
debated
whether
amyloid
angiopathy
plays
a
causative
role
in
Alzheimer
's
disease
,
but
it
is
strongly
correlated
with
the
presence
of
cerebral
plaques
.
In
this
review
,
the
clinical
spectrum
of
cerebral
amyloid
angiopathy
will
be
described
,
based
on
retrospective
studies
from
the
literature
.
Hereditary
cerebral
hemorrhage
with
amyloidosis
(
Dutch
)
will
be
presented
as
a
'
prospective
'
model
to
study
the
clinical
effects
of
amyloid
angiopathy
.
Diseases
Validation
Diseases presenting
"and leukoencephalopathy"
symptom
hereditary cerebral hemorrhage with amyloidosis
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