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Corticospinal tract and pontocerebellar fiber of central pontine myelinolysis.
[locked-in syndrome]
Central
pontine
myelinolysis
is
a
rare
neurologic
disorder
that
is
defined
by
demyelination
of
longitudinally
descending
tracts
and
transversly
crossing
fibers
in
the
basis
pontis
.
Frequently
observed
clinical
manifestations
of
this
disorder
include
sudden
weakness
,
dysphagia
,
loss
of
consciouness
and
locked-
in
syndrome
.
However
,
there
have
been
a
few
studies
that
reported
a
benign
course
of
this
disease
,
which
include
cerebellar
signs
,
such
as
ataxia
,
intention
tremor
,
and
dysarthria
.
Here
we
report
on
a
53
-
year
-old
male
with
a
history
of
liver
cirrhosis
who
showed
the
cerebellar
type
of
central
pontine
myelinolysis
.
The
patient
was
diagnosed
with
central
pontine
myelinolysis
based
on
clinical
presentations
and
magnetic
resonance
imaging
findings
after
a
liver
transplantation
.
Conventional
magenetic
resonance
imaging
(
MRI
)
revealed
the
preservation
of
the
corticospinal
tract
and
abnormal
pontocerebellar
fibers
.
However
,
these
findings
were
not
sufficient
to
define
the
pathophysiology
of
our
patient
.
Electrophysiologic
analysis
and
diffusion
tensor
imaging
(
DTI
)
were
performed
to
investigate
cerebellar
signs
in
this
case
.
Delayed
central
motor
conduction
time
(
CMCT
)
to
the
tibialis
anterior
muscle
with
transcranial
magnetic
stimulation
(
TMS
)
was
observed
,
which
indicated
demyelination
of
the
corticospinal
tract
.
Also
,
diffusion
tensor
imaging
showed
abnormal
pontocerebellar
fibers
,
which
might
have
been
caused
by
cerebellar
dysfunction
in
our
patient
.
A
combination
of
TMS
and
DTI
was
also
used
to
determine
the
pathophysiology
of
this
disease
.
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