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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
"broad spectrum"
symptom
adrenomyeloneuropathy
cushing syndrome
cystinuria
dentinogenesis imperfecta
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
focal myositis
hereditary cerebral hemorrhage with amyloidosis
hodgkin lymphoma, classical
kabuki syndrome
kallmann syndrome
krabbe disease
neonatal adrenoleukodystrophy
omenn syndrome
pendred syndrome
pleomorphic liposarcoma
triple a syndrome
waldenström macroglobulinemia
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