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Clinical features of Japanese patients with inclusion body myositis.
[inclusion body myositis]
The
incidence
of
sporadic
inclusion
body
myositis
(
sIBM
)
has
been
much
lower
in
Japanese
than
in
Western
populations
.
Because
of
a
few
reports
on
Asian
populations
,
it
is
unclear
whether
the
clinical
characteristics
of
sIBM
are
identical
in
Caucasian
and
Japanese
patients
.
We
compared
18
patients
with
sIBM
,
divided
into
3
groups
by
age-of-onset
,
with
previous
cohort
studies
.
We
calculated
the
ΔIBM
functional
rating
scale
/
time
duration
(
ΔIBMFRS
/
Δtime
)
as
an
index
of
functional
disability
progression
.
Patients
'
electrophysiology
was
analyzed
in
relation
to
their
clinical
characteristics
.
The
cohort
was
83
.
3
%
male
and
showed
uniform
initial
muscle
weakness
in
the
lower
and
/
or
upper
limbs
.
An
older
age-
at
-onset
was
associated
with
a
more
rapid
progression
,
and
patients
with
a
longer
duration
frequently
showed
F-
wave
abnormalities
and
findings
of
chronic
denervation
.
The
clinical
characteristics
of
sIBM
were
relatively
homogeneous
beyond
the
ethnic
differences
.
Aging
might
be
a
synergistic
factor
for
the
progression
of
sIBM
pathology
.
Diseases
Validation
Diseases presenting
"and patients with a longer duration frequently showed f-wave abnormalities and findings of chronic denervation"
symptom
inclusion body myositis
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