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The clinical spectrum of neuralgic amyotrophy in 246 cases.
[neuralgic amyotrophy]
We
investigated
the
symptoms
,
course
and
prognosis
of
neuralgic
amyotrophy
(
NA
)
in
a
large
group
of
patients
with
idiopathic
neuralgic
amyotrophy
(
INA
,
n
=
199
)
and
hereditary
neuralgic
amyotrophy
(
HNA
,
n
=
47
)
to
gain
more
insight
into
the
broad
clinical
spectrum
of
the
disorder
.
Several
findings
from
earlier
smaller-scale
studies
were
tested
,
and
for
the
first
time
the
potential
differences
between
the
hereditary
and
idiopathic
phenotypes
and
between
males
and
females
were
explored
.
Generally
,
the
course
of
the
pain
manifests
itself
in
three
consecutive
phases
with
an
initial
severe
,
continuous
pain
lasting
for
approximately
4
weeks
on
average
.
Sensory
involvement
was
quite
common
and
found
in
78
.
4
%
of
patients
but
was
clinically
less
impairing
than
the
initial
pain
and
subsequent
paresis
.
As
a
typically
patchy
disorder
NA
can
affect
almost
any
nerve
in
the
brachial
plexus
,
although
damage
in
the
upper
and
middle
trunk
distribution
with
involvement
of
the
long
thoracic
and
/
or
suprascapular
nerve
occurred
most
frequently
(
71
.
1
%
)
.
We
found
no
correlation
between
the
distribution
of
motor
and
sensory
symptoms
.
In
INA
recurrent
attacks
were
found
in
26
.
1
%
of
the
patients
during
an
average
6
year
follow-up
.
HNA
patients
had
an
earlier
onset
(
28
.
4
versus
41
.
3
years
)
,
more
attacks
(
mean
3
.
5
versus
1
.
5
)
and
more
frequent
involvement
of
nerves
outside
the
brachial
plexus
(
55
.
8
versus
17
.
3
%
)
than
INA
patients
,
and
a
more
severe
maximum
paresis
,
with
a
subsequent
poorer
functional
outcome
.
In
males
the
initial
pain
tended
to
last
longer
than
it
did
in
females
(
45
versus
23
days
)
.
In
females
the
middle
or
lower
parts
of
the
brachial
plexus
were
involved
more
frequently
(
23
.
1
versus
10
.
5
%
in
males
)
,
and
their
functional
outcome
was
worse
.
Overall
recovery
was
less
favourable
than
usually
assumed
,
with
persisting
pain
and
paresis
in
approximately
two
-thirds
of
the
patients
who
were
followed
for
3
years
or
more
.
Diseases
Validation
Diseases presenting
"first time"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
adrenomyeloneuropathy
alpha-thalassemia
aniridia
aromatase deficiency
canavan disease
carcinoma of the gallbladder
cholangiocarcinoma
classical phenylketonuria
congenital adrenal hyperplasia
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dentin dysplasia
dentinogenesis imperfecta
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
gm1 gangliosidosis
harlequin ichthyosis
heparin-induced thrombocytopenia
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
hydrocephalus with stenosis of the aqueduct of sylvius
junctional epidermolysis bullosa
kabuki syndrome
kallmann syndrome
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
monosomy 21
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
omenn syndrome
oral submucous fibrosis
papillon-lefèvre syndrome
pendred syndrome
phenylketonuria
primary effusion lymphoma
primary hyperoxaluria type 1
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
trochlear dysplasia
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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