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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
"muscle weakness"
symptom
alexander disease
canavan disease
coats disease
cohen syndrome
cystinuria
familial mediterranean fever
focal myositis
inclusion body myositis
neuralgic amyotrophy
pyomyositis
severe combined immunodeficiency
systemic capillary leak syndrome
thoracic outlet syndrome
triple a syndrome
von hippel-lindau disease
This symptom has already been validated