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[Lewis-Sumner syndrome presenting unilateral quadriceps amyotrophy as an initial symptom].
[focal myositis]
We
report
a
55
-
year
-old
man
with
a
chief
complaint
of
wasting
and
weakness
of
the
left
quadriceps
muscle
.
At
age
54
,
he
noticed
difficulty
in
running
and
weakness
in
the
left
thigh
,
which
gradually
progressed
.
On
the
first
admission
to
our
hospital
,
based
on
the
nerve
conduction
studies
(
NCS
)
,
the
muscle
biopsy
findings
showing
neurologenic
changes
,
and
no
abnormality
of
spinal
MRI
,
we
diagnosed
as
unilateral
quadriceps
amyotrophy
,
which
resulted
from
an
atypical
form
of
spinal
progressive
muscular
atrophy
.
One
year
later
,
he
showed
the
bilateral
hand
weakness
,
conduction
blocks
on
the
right
median
and
ulnar
nerves
by
NCS
,
and
the
presence
of
serum
anti-
GM
1
antibody
.
From
these
findings
,
Lewis-
Sumner
syndrome
was
diagnosed
.
The
therapy
of
high
-dose
intravenous
immunoglobulin
moderately
improved
his
symptoms
.
The
clinical
symptoms
of
quadriceps
amyotrophy
is
produced
by
various
disorders
including
spinal
progressive
muscular
atrophy
,
spinal
extradural
arachnoid
cyst
,
rimmed
vacuole
myopathy
,
Becker
dystrophy
,
limb
-girdle
dystrophy
,
and
focal
myositis
.
However
,
there
have
been
no
reports
of
a
case
of
Lewis-
Sumner
syndrome
.
It
is
important
to
consider
Lewis-
Sumner
syndrome
in
the
differential
diagnosis
of
quadriceps
amyotrophy
.
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