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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
"weakness"
symptom
achondroplasia
adrenomyeloneuropathy
alexander disease
aniridia
aromatase deficiency
coats disease
congenital toxoplasmosis
epidermolysis bullosa simplex
esophageal adenocarcinoma
familial hypocalciuric hypercalcemia
familial mediterranean fever
focal myositis
gm1 gangliosidosis
hydrocephalus with stenosis of the aqueduct of sylvius
inclusion body myositis
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
neuralgic amyotrophy
pendred syndrome
pleomorphic liposarcoma
pyomyositis
pyruvate dehydrogenase deficiency
sneddon syndrome
systemic capillary leak syndrome
thoracic outlet syndrome
triple a syndrome
typhoid
von hippel-lindau disease
waldenström macroglobulinemia
werner syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated
