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Isolated neck extensor myopathy: is it responsive to immunotherapy?
[focal myositis]
To
determine
if
isolated
neck
extensor
myopathy
(
INEM
)
is
responsive
to
immunosuppressive
treatment
.
We
retrospectively
reviewed
charts
of
patients
with
INEM
from
2002
to
2008
to
identify
patients
and
determine
the
response
to
immunomodulatory
therapy
.
Clinical
,
electrodiagnostic
,
histologic
,
and
radiographic
data
were
reviewed
.
Four
patients
were
identified
during
the
study
period
.
Three
were
women
.
The
age
of
onset
of
neck
extensor
weakness
ranged
from
58
to
78
years
.
Serum
creatine
kinase
levels
were
within
normal
limits
in
all
patients
.
None
had
clinical
,
laboratory
,
or
electrophysiological
findings
to
suggest
a
generalized
neuromuscular
disorder
.
On
electrodiagnostic
studies
,
all
patients
had
myopathic
changes
with
or
without
irritative
features
in
cervical
paraspinal
muscles
.
No
inflammation
was
present
on
muscle
biopsy
from
three
of
the
patients
.
All
patients
received
one
or
more
immunosuppressive
agents
.
Neck
strength
improved
by
1
point
or
greater
on
the
Medical
Research
Council
scale
in
all
subjects
with
a
peak
response
observed
between
3
and
6
months
after
treatment
initiation
.
A
trial
of
immunosuppressive
agents
should
be
offered
to
patients
with
INEM
because
a
subset
will
improve
.
Rigorously
defined
,
INEM
is
a
noninflammatory
myopathy
.
However
,
a
focal
myositis
could
be
missed
on
muscle
biopsy
and
may
explain
the
favorable
response
to
treatment
.
Diseases
Validation
Diseases presenting
"myopathy"
symptom
coats disease
cushing syndrome
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
familial mediterranean fever
focal myositis
homocystinuria without methylmalonic aciduria
inclusion body myositis
junctional epidermolysis bullosa
lymphangioleiomyomatosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
pyruvate dehydrogenase deficiency
This symptom has already been validated