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A rare cause of neuromuscular scoliosis: Alexander disease.
[alexander disease]
Alexander
disease
belongs
to
a
group
of
progressive
neurological
disorders
in
which
the
destruction
of
white
matter
in
the
brain
is
accompanied
by
the
formation
of
fibrous
,
eosinophilic
deposits
known
as
Rosenthal
fibers
.
Spinal
deformity
rarely
accompanies
this
disease
.
The
aim
of
this
report
is
to
present
a
patient
with
juvenile
-onset
Alexander
disease
,
who
also
had
progressive
neuromuscular
type
scoliosis
requiring
surgical
stabilization
.
A
13
-
year
-old
male
presented
with
bilateral
weakness
in
both
lower
extremities
and
scoliosis
.
Results
of
an
examination
of
the
spine
showed
a
left
thoracic
scoliosis
with
prominent
left
paraspinous
prominence
and
elevation
of
the
ipsilateral
shoulder
.
Spinal
fusion
with
rigid
internal
stabilizing
instrumentation
was
selected
for
surgical
treatment
of
the
scoliosis
.
The
fusion
area
was
to
be
from
T
2
to
L
4
.
He
was
instrumented
with
pedicle
screw
fixation
system
,
and
he
underwent
fusion
with
an
allogenous
bone
graft
.
Satisfactory
correction
of
the
sagittal
and
coronal
plane
deformity
was
achieved
,
reducing
the
scoliosis
to
14
degrees
.
At
the
5
-
year
follow-up
,
results
of
a
clinical
examination
showed
a
marked
improvement
in
truncal
balance
and
walking
ability
.
The
patient
had
a
rapidly
progressive
scoliosis
and
severe
decompensation
requiring
surgical
stabilization
.
The
scoliosis
behaved
in
a
manner
similar
to
that
of
neuromuscular
scoliosis
.
Therefore
,
more
aggressive
treatment
was
warranted
to
prevent
decompensation
.
For
that
reason
,
posterior
long
segment
(
T
2
-
L
4
)
pedicle
screw
instrumentation
and
fusion
was
performed
for
surgical
treatment
.
Diseases
Validation
Diseases presenting
"coronal plane deformity"
symptom
alexander disease
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