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Dutch hereditary cerebral amyloid angiopathy: structural lesions and apolipoprotein E genotype.
[hereditary cerebral hemorrhage with amyloidosis]
Hereditary
cerebral
hemorrhage
with
amyloidosis
-
Dutch
type
is
caused
by
a
mutation
at
codon
693
of
the
beta
amyloid
precursor
protein
gene
.
The
disease
is
clinically
characterized
by
strokes
and
dementia
.
In
addition
to
cerebral
plaques
,
cerebral
amyloid
angiopathy
is
the
pathological
hallmark
.
We
investigated
the
correlation
between
radiological
(
white
matter
hyperintensities
and
focal
lesions
on
magnetic
resonance
images
)
and
pathological
lesions
(
cerebrovascular
amyloid
angiopathy
and
plaques
)
and
the
apolipoprotein
E
genotype
in
patients
with
the
disease
.
Twenty
-
five
patients
were
studied
using
magnetic
resonance
imaging
,
and
brain
tissue
from
8
patients
was
studied
histopathologically
.
Neither
the
white
matter
hyperintensity
scores
nor
the
number
of
focal
lesions
on
magnetic
resonance
images
were
associated
with
the
presence
of
an
epsilon
4
allele
.
Nor
was
a
correlation
found
between
the
number
and
type
of
plaques
and
the
apolipoprotein
E
genotype
.
All
patients
had
severe
amyloid
angiopathy
in
all
cortical
areas
investigated
.
This
study
showed
that
the
apolipoprotein
E
genotype
does
not
modulate
amyloid-related
structural
lesions
in
hereditary
cerebral
hemorrhage
with
amyloidosis
of
the
Dutch
type
.
Diseases
Validation
Diseases presenting
"hereditary cerebral hemorrhage"
symptom
hereditary cerebral hemorrhage with amyloidosis
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