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Novel diffusion tensor imaging findings in Krabbe disease.
[krabbe disease]
Krabbe
disease
is
a
lysosomal
disorder
that
primarily
affects
myelin
.
Diffusion
tensor
imaging
(
DTI
)
provides
quantitative
information
about
the
white
matter
organization
and
integrity
.
Radial
diffusivity
(
RD
)
reflects
myelin
injury
selectively
.
To
report
on
quantitative
DTI
findings
(
including
axial
diffusivity
(
AD
)
and
RD
,
not
previously
reported
)
in
two
children
with
Krabbe
disease
compared
to
controls
.
A
quantitative
region
of
interest
(
ROI
)
based
DTI
analysis
was
performed
for
the
patients
and
age-
and
gender-matched
controls
.
Fractional
anisotropy
(
FA
)
,
mean
diffusivity
,
AD
and
RD
values
as
well
as
variation
ratios
between
the
patients
'
and
controls
'
values
were
calculated
for
nine
brain
regions
.
Two
boys
with
Krabbe
disease
were
included
in
this
study
.
DTI
data
were
acquired
at
the
ages
of
6
.
25
years
and
6
.
5
months
.
For
all
regions
,
FA
ratios
were
negative
,
while
RD
and
MD
ratios
positive
.
The
most
elevated
variation
ratios
were
found
for
RD
.
Variation
ratios
were
greater
in
the
centrum
semiovale
,
corpus
callosum
,
and
middle
cerebellar
peduncles
than
in
other
anatomical
regions
,
especially
in
the
older
patient
in
comparison
with
the
younger
patient
.
The
AD
ratios
,
however
,
were
much
lower
and
close
to
zero
.
DTI
allows
a
quantitative
evaluation
of
white
matter
damage
in
Krabbe
disease
.
RD
seems
to
be
the
most
sensitive
DTI
parameter
in
agreement
with
the
histopathological
findings
in
Krabbe
disease
,
a
primary
myelin
disorder
.
This
may
be
important
in
the
early
detection
of
the
onset
of
demyelination
.
Diseases
Validation
Diseases presenting
"white matter damage"
symptom
hereditary cerebral hemorrhage with amyloidosis
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
krabbe disease
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