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White Matter Edema at the Early Stage of Cerebral Autosomal-Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy.
[cadasil]
Recently
,
in
a
mouse
model
of
cerebral
autosomal-dominant
arteriopathy
with
subcortical
infarcts
and
leukoencephalopathy
,
a
monogenic
cerebral
small
vessel
disease
,
intramyelinic
edema
was
detected
in
the
white
matter
(
WM
)
early
during
the
course
of
the
disease
.
We
hypothesized
that
if
this
mechanism
holds
true
in
patients
,
it
would
translate
in
larger
WM
volume
.
We
aimed
to
measure
WM
volume
in
patients
with
cerebral
autosomal-dominant
arteriopathy
with
subcortical
infarcts
and
leukoencephalopathy
in
comparison
with
age-
and
sex-matched
controls
,
along
with
the
ratio
of
cortical
surface
area
to
the
volume
of
brain
hemispheres
as
an
indirect
measure
that
should
be
reduced
in
patients
.
Twenty
patients
at
the
early
stage
of
the
disease
(
Mini
Mental
State
Examination
>
24
and
modified
Rankin
scale
≤
1
)
and
27
age-
and
sex-matched
controls
had
high
-quality
3
-
Tesla
3
DT
1
MRI
acquisitions
.
Volumes
of
brain
hemispheres
and
of
WM
were
determined
.
The
ratio
of
cortical
surface
area
to
the
volume
of
brain
hemispheres
was
evaluated
as
a
proxy
of
underlying
WM
volume
.
Patients
had
larger
volumes
of
WM
than
controls
(
patients
:
479
.
4
±
71
.
7
;
controls
:
463
.
9
±
44
.
2
;
P
=
0
.
03
)
.
They
presented
a
lower
cortical
surface
area
and
cortical
volume
leading
to
a
lower
ratio
of
cortical
surface
area
to
the
volume
of
brain
hemispheres
(
patients
:
15
.
7
±
0
.
7
;
controls
:
16
.
1
±
0
.
5
;
P
=
0
.
004
)
.
Volume
of
WM
tended
to
be
associated
with
that
of
WM
hyperintensities
(
P
=
0
.
06
)
.
Patients
with
cerebral
autosomal-dominant
arteriopathy
with
subcortical
infarcts
and
leukoencephalopathy
have
larger
WM
volume
than
age-
and
sex-matched
controls
,
a
finding
compatible
with
the
hypothesis
of
intramyelinic
edema
as
observed
recently
in
mice
.
