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Cerebral hemorrhages in CADASIL: report of four cases and a brief review.
[cadasil]
Cerebral
autosomal
dominant
arteriopathy
with
subcortical
infarcts
and
leukoencephalopathy
(
CADASIL
)
is
an
inherited
cerebral
small
vessel
disease
,
clinically
characterized
by
migraine
,
recurrent
transient
ischemic
attacks
or
strokes
,
psychiatric
disorders
and
cognitive
decline
.
Strokes
are
typically
ischemic
,
while
hemorrhagic
events
have
been
only
sporadically
described
.
However
,
cerebral
microbleeds
have
been
found
in
31
-
69
%
of
CADASIL
patients
.
We
describe
four
unrelated
CADASIL
patients
who
had
hemorrhagic
strokes
.
We
also
briefly
review
the
literature
on
intracerebral
hemorrhage
(
ICH
)
in
CADASIL
.
Three
patients
had
a
thalamo-capsular
hemorrhage
(
age
at
onset
:
54
,
67
,
77
)
and
one
of
these
had
a
second
hemispheric
cerebellar
hemorrhage
.
Another
patient
experienced
an
interpeduncular
cistern
subarachnoid
hemorrhage
when
he
was
39
.
None
of
these
patients
was
receiving
antiplatelets
,
anticoagulants
or
statins
at
the
time
of
hemorrhage
;
all
were
hypertensive
.
NOTCH
3
gene
analysis
revealed
mutations
on
exons
14
,
22
(
two
patients
presenting
the
same
mutation
)
,
and
24
.
MRI
signs
of
previous
hemorrhages
were
present
in
all
these
patients
.
Hemorrhagic
stroke
can
occur
in
CADASIL
similarly
to
sporadic
cerebral
small
vessel
diseases
;
this
finding
expands
the
phenotype
of
the
disease
.
A
diagnosis
of
CADASIL
should
probably
be
considered
also
in
patients
with
ICH
.
These
data
bear
potential
implications
in
terms
of
need
of
better
control
of
risk
factors
,
particularly
hypertension
,
and
raise
relevant
questions
about
the
use
of
antiplatelets
as
prevention
measures
in
CADASIL
patients
.
Diseases
Validation
Diseases presenting
"transient ischemic attacks"
symptom
cadasil
fabry disease
sneddon syndrome
This symptom has already been validated