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Radiologic and neuropathologic findings in patients in a family with dentatorubral-pallidoluysian atrophy.
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We
describe
the
cases
of
2
patients
,
a
father
and
his
son
,
with
DRPLA
who
underwent
MR
examinations
prior
to
death
and
in
whom
postmortem
examinations
of
the
brain
were
obtained
.
MR
imaging
findings
had
the
following
features
:
1
)
atrophy
of
the
cerebellum
and
brain
stem
were
the
common
findings
,
2
)
high
-signal-intensity
lesions
in
the
cerebral
white
matter
and
brain
stem
were
observed
on
T
2
-
weighted
images
in
the
patient
with
adult-onset
DRPLA
,
3
)
signal-intensity
changes
in
the
cerebral
white
matter
were
restricted
to
the
periventricular
white
matter
in
the
patient
with
juvenile
-onset
DRPLA
,
but
these
changes
appear
in
the
advanced
stage
,
and
4
)
progressive
cerebral
atrophy
was
more
marked
in
the
patient
with
juvenile
-onset
DRPLA
.
In
the
patients
with
DRPLA
,
the
abnormal
high
signal
intensity
of
the
cerebral
white
matter
or
brain
stem
on
MR
images
reflect
the
loss
of
myelinated
fibers
.
Cerebral
atrophy
mainly
reflects
atrophy
of
the
neuropile
.
