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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
"liver cirrhosis"
symptom
allergic bronchopulmonary aspergillosis
benign recurrent intrahepatic cholestasis
cholangiocarcinoma
erythropoietic protoporphyria
hirschsprung disease
locked-in syndrome
papillon-lefèvre syndrome
primary effusion lymphoma
This symptom has already been validated