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Sporadic and familial cerebral amyloid angiopathies.
[hereditary cerebral hemorrhage with amyloidosis]
Cerebral
amyloid
angiopathy
(
CAA
)
is
the
term
used
to
describe
deposition
of
amyloid
in
the
walls
of
arteries
,
arterioles
and
,
less
often
,
capillaries
and
veins
of
the
central
nervous
system
.
CAAs
are
an
important
cause
of
cerebral
hemorrhage
and
may
also
result
in
ischemic
lesions
and
dementia
.
A
number
of
amyloid
proteins
are
known
to
cause
CAA
.
The
most
common
sporadic
CAA
,
caused
by
A
beta
deposition
,
is
associated
with
aging
and
is
a
common
feature
of
Alzheimer
disease
(
AD
)
.
CAA
occurs
in
several
familial
conditions
,
including
hereditary
cerebral
hemorrhage
with
amyloidosis
of
Icelandic
type
caused
by
deposition
of
mutant
cystatin
C
,
hereditary
cerebral
hemorrhage
with
amyloidosis
Dutch
type
and
familial
AD
with
deposition
of
either
A
beta
variants
or
wild-
type
A
beta
,
the
transthyretin
-related
meningo-
vascular
amyloidoses
,
gelsolin
as
well
as
familial
prion
disease
-related
CAAs
and
the
recently
described
BRI
2
gene
-related
CAAs
in
familial
British
dementia
and
familial
Danish
dementia
.
This
review
focuses
on
the
morphological
,
biochemical
,
and
genetic
aspects
as
well
as
the
clinical
significance
of
CAAs
with
special
emphasis
on
the
BRI
2
gene
-related
cerebrovascular
amyloidoses
.
We
also
discuss
data
relevant
to
the
pathomechanism
of
the
different
forms
of
CAA
with
an
emphasis
on
the
most
common
A
beta
-related
types
.
Diseases
Validation
Diseases presenting
"central nervous system"
symptom
22q11.2 deletion syndrome
adrenomyeloneuropathy
alexander disease
aniridia
aromatase deficiency
canavan disease
child syndrome
classical phenylketonuria
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cystinuria
dracunculiasis
erdheim-chester disease
fabry disease
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
hirschsprung disease
hodgkin lymphoma, classical
kabuki syndrome
kallmann syndrome
kindler syndrome
krabbe disease
lamellar ichthyosis
legionellosis
liposarcoma
malignant atrophic papulosis
monosomy 21
neonatal adrenoleukodystrophy
phenylketonuria
proteus syndrome
scrub typhus
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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