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Descriptive analysis of the Boston criteria applied to a Dutch-type cerebral amyloid angiopathy population.
[hereditary cerebral hemorrhage with amyloidosis]
Validation
of
the
Boston
criteria
for
the
in
vivo
diagnosis
of
cerebral
amyloid
angiopathy
(
CAA
)
is
challenging
,
because
noninvasive
diagnostic
tests
do
not
exist
.
Hereditary
cerebral
hemorrhage
with
amyloidosis
-
Dutch
type
is
an
accepted
monogenetic
model
of
CAA
and
diagnosis
can
be
made
with
certainty
based
on
DNA
analysis
.
The
aim
of
this
study
was
to
analyze
and
refine
the
existing
Boston
criteria
in
patients
with
hereditary
cerebral
hemorrhage
with
amyloidosis
-
Dutch
type
.
We
performed
T
2
*
-
weighted
MRI
in
27
patients
with
hereditary
cerebral
hemorrhage
with
amyloidosis
-
Dutch
type
to
assess
the
presence
and
location
of
microbleeds
,
intracranial
hemorrhages
,
and
superficial
siderosis
.
Using
the
Boston
criteria
,
subjects
were
categorized
as
having
:
no
hemorrhages
,
possible
CAA
,
probable
CAA
,
and
hemorrhagic
lesions
not
qualifying
for
CAA
.
The
sensitivity
of
the
Boston
criteria
was
calculated
separately
using
intracranial
hemorrhages
only
and
using
intracranial
hemorrhages
and
microbleeds
.
The
sensitivity
of
the
Boston
criteria
for
probable
CAA
increased
from
48
%
to
63
%
when
microbleeds
were
included
.
For
symptomatic
subjects
only
,
the
sensitivity
was
100
%
.
No
hemorrhages
were
identified
in
the
deep
white
matter
,
basal
ganglia
,
thalamus
,
or
brainstem
.
Superficial
siderosis
,
observed
in
6
patients
,
did
not
increase
the
sensitivity
of
the
Boston
criteria
in
our
study
group
.
Our
data
show
that
using
T
2
*
-
weighted
MRI
and
including
microbleeds
increase
the
sensitivity
of
the
Boston
criteria
.
The
exclusion
of
hemorrhages
in
the
deep
white
matter
,
basal
ganglia
,
thalamus
,
and
brainstem
does
not
lower
the
sensitivity
of
the
Boston
criteria
.
Diseases
Validation
Diseases presenting
"cerebral hemorrhage"
symptom
cohen syndrome
hereditary cerebral hemorrhage with amyloidosis
sneddon syndrome
This symptom has already been validated