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Intravenous immunoglobulin (IVIg) with methylprednisolone pulse therapy for motor impairment of neuralgic amyotrophy: clinical observations in 10 cases.
[neuralgic amyotrophy]
Neuralgic
amyotrophy
(
NA
)
is
a
distinct
peripheral
nervous
system
disorder
characterized
by
attacks
of
acute
neuropathic
pain
and
rapid
multifocal
weakness
and
atrophy
unilaterally
in
the
upper
limb
.
The
current
hypothesis
is
that
the
episodes
are
caused
by
an
immune-mediated
response
to
the
brachial
plexus
,
however
,
therapeutic
strategies
for
NA
have
not
been
well
established
.
We
retrospectively
reviewed
15
case
series
of
NA
;
10
of
the
15
patients
received
intravenous
immunoglobulin
(
IVIg
)
with
methylprednisolone
pulse
therapy
(
MPPT
)
and
9
of
these
0
patients
showed
clinical
improvement
of
motor
impairment
.
Our
clinical
observations
do
not
contradict
the
possibility
that
IVIg
with
MPPT
may
be
one
of
the
potential
therapeutics
for
NA
,
however
the
efficacy
remains
to
be
established
.
Further
confirmatory
trials
are
needed
in
patients
with
various
clinical
severities
and
phases
of
NA
.
Further
basic
research
and
confirmatory
trials
should
be
performed
to
survey
the
efficacy
of
such
immunomodulation
therapy
for
NA
.
Diseases
Validation
Diseases presenting
"pain"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
adrenomyeloneuropathy
aniridia
aromatase deficiency
carcinoma of the gallbladder
cholangiocarcinoma
coats disease
congenital diaphragmatic hernia
congenital toxoplasmosis
cushing syndrome
cutaneous mastocytosis
cystinuria
dedifferentiated liposarcoma
dentin dysplasia
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
focal myositis
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
junctional epidermolysis bullosa
kabuki syndrome
kindler syndrome
lamellar ichthyosis
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
neuralgic amyotrophy
oligodontia
oral submucous fibrosis
papillon-lefèvre syndrome
phenylketonuria
pleomorphic liposarcoma
primary hyperoxaluria type 1
proteus syndrome
pyomyositis
scrub typhus
sneddon syndrome
systemic capillary leak syndrome
thoracic outlet syndrome
trochlear dysplasia
typhoid
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
wolf-hirschhorn syndrome
This symptom has already been validated