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Inclusion-body myositis presenting with facial diplegia.
[inclusion body myositis]
The
hallmark
clinical
presentation
of
inclusion-body
myositis
(
IBM
)
is
slowly
progressive
weakness
that
characteristically
affects
the
quadriceps
and
finger
and
wrist
finger
flexor
muscles
.
Facial
weakness
can
also
occur
,
but
it
is
typically
mild
and
not
a
prominent
finding
.
We
describe
the
clinical
features
,
laboratory
investigations
,
and
muscle
biopsy
findings
in
a
58
-
year
old
man
who
presented
with
a
6
-
year
history
of
marked
progressive
symmetrical
facial
weakness
.
Examination
also
showed
shoulder
abduction
and
hip
extensor
weakness
.
The
patient
's
serum
creatine
kinase
level
was
655
U
/
L
,
and
electromyography
showed
fibrillation
potentials
and
myopathic
motor
unit
potentials
.
A
biopsy
specimen
of
the
left
biceps
muscle
was
pathognomonic
for
IBM
.
T
his
patient
did
not
have
a
typical
presentation
for
IBM
but
rather
fulfilled
the
pathological
criteria
for
IBM
.
To
our
knowledge
,
facial
diplegia
has
not
been
reported
previously
as
a
presenting
manifestation
of
IBM
.
Diseases
Validation
Diseases presenting
"myositis"
symptom
focal myositis
inclusion body myositis
malignant atrophic papulosis
pyomyositis
systemic capillary leak syndrome
This symptom has already been validated