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Childhood cerebral X-linked adrenoleukodystrophy: diffusion tensor imaging measurements for prediction of clinical outcome after hematopoietic stem cell transplantation.
[x-linked adrenoleukodystrophy]
DTI
in
cerebral
X-
linked
adrenoleukodystrophy
may
demonstrate
abnormalities
in
both
affected
and
nonaffected
WM
;
these
values
have
not
been
studied
serially
after
hematopoietic
stem
cell
transplantation
.
The
purpose
of
this
study
was
to
study
pretransplant
and
posttransplant
DTI
parameters
serially
and
ultimately
to
determine
the
ability
of
pretransplant
DTI
parameters
to
predict
clinical
outcome
after
HSCT
in
children
with
ALD
.
Eight
patients
with
posterior
-pattern
cerebral
ALD
underwent
DTI
at
3
T
before
HSCT
(
T
0
)
,
at
30
-
60
days
(
T
1
)
,
90
-
120
days
(
T
2
)
,
180
days
(
T
3
)
,
and
1
year
(
T
4
)
after
HSCT
.
FA
and
MD
were
serially
measured
in
19
regions
,
and
these
measurements
were
compared
with
those
in
control
patients
.
MR
imaging
severity
(
Loes
)
scores
were
recorded
.
Correlations
were
performed
between
DTI
parameters
and
Loes
scores
,
neurologic
function
scores
,
and
several
neuropsychologic
scores
.
Both
FA
and
MD
in
subjects
differed
significantly
from
that
in
controls
at
nearly
every
time
point
within
cerebellar
WM
,
callosal
splenium
,
and
parieto-
occipital
WM
;
FA
alone
was
significantly
different
at
each
time
point
within
the
optic
radiations
,
lateral
geniculate
,
and
the
Meyer
loop
(
P
<
.
05
)
.
Loes
scores
at
T
0
correlated
strongly
with
each
clinical
score
at
T
4
(
r
=
0
.
771
-
0
.
986
,
P
<
.
05
)
.
The
only
significant
DTI
correlation
at
T
0
with
a
clinical
score
at
T
4
was
callosal
body
FA
with
adaptive
function
(
r
=
0
.
976
,
P
<
.
001
)
.
Correlating
the
change
in
DTI
values
with
change
in
NFS
(
change
between
T
0
and
T
4
)
showed
that
only
ΔMD
within
the
optic
radiations
correlated
strongly
with
ΔNFS
(
r
=
0
.
903
,
P
<
.
05
)
.
DTI
values
at
T
0
were
generally
poor
predictors
of
outcome
at
1
year
,
whereas
Loes
scores
were
generally
good
predictors
.
ΔMD
within
the
optic
radiations
strongly
correlates
with
ΔNFS
over
that
year
.
In
addition
,
certain
normal-appearing
regions
,
such
as
cerebellar
WM
,
may
have
DTI
abnormalities
before
HSCT
that
persist
after
HSCT
.
Diseases
Validation
Diseases presenting
"change in nfs"
symptom
x-linked adrenoleukodystrophy
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