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Deep Brain Stimulation and Dantrolene for Secondary Dystonia in X-Linked Adrenoleukodystrophy.
[x-linked adrenoleukodystrophy]
Deep
brain
stimulation
(
DBS
)
has
been
used
to
treat
secondary
dystonias
caused
by
inborn
errors
of
metabolism
with
varying
degrees
of
effectiveness
.
Here
we
report
for
the
first
time
the
application
of
DBS
as
treatment
for
secondary
dystonia
in
a
22
-
year
-old
male
with
X-
linked
adrenoleukodystrophy
(
X-
ALD
)
.
The
disease
manifested
at
age
6
with
ADHD
,
tics
,
and
dystonic
gait
,
and
deteriorated
to
loss
of
ambulation
by
age
11
,
and
speech
difficulties
,
seizures
,
and
characteristic
adrenal
insufficiency
by
age
16
.
DBS
in
the
globus
pallidus
internus
was
commenced
at
age
18
.
However
,
after
25
months
,
no
improvement
in
dystonia
was
observed
(
Burke-
Fahn-
Marsden
(
BFM
)
scores
of
65
.
5
and
62
and
disability
scores
of
28
and
26
,
pre-
and
post-
DBS
,
respectively
)
and
the
DBS
device
was
removed
.
Treatment
with
dantrolene
reduced
skeletal
muscle
tone
and
improved
movement
(
Global
Dystonia
Rating
Scores
from
5
to
1
and
BFM
score
42
)
.
Therefore
,
we
conclude
that
DBS
was
a
safe
but
ineffective
intervention
in
our
case
with
long
-standing
dystonia
,
whereas
treatment
of
spasticity
with
dantrolene
did
improve
the
movement
disorder
in
this
young
man
with
X-
ALD
.
Diseases
Validation
Diseases presenting
"tics"
symptom
sneddon syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated