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Hourglass-like constrictions of peripheral nerve in the upper extremity: a clinical review and pathological study.
[neuralgic amyotrophy]
The
development
of
an
hourglass-like
constriction
in
the
nerve
is
rare
,
and
its
origin
is
unknown
.
Its
clinical
manifestations
are
not
well
documented
,
and
the
treatment
protocol
has
not
been
established
.
To
identify
the
cause
,
presentation
,
and
possible
treatment
for
patients
with
nerve
palsies
secondary
to
an
hourglass-like
constriction
in
the
affected
nerves
.
Patients
presenting
with
peripheral
nerve
palsy
caused
by
an
hourglass-like
constriction
of
nerves
were
retrospectively
investigated
in
2
hand
centers
.
The
patients
'
presentation
and
neurological
findings
were
reviewed
,
and
the
immunohistochemistry
of
excised
specimens
was
studied
.
Forty
-
two
patients
who
presented
with
47
nerve
palsies
were
examined
.
Forty
-
one
patients
experienced
a
sudden
onset
of
pain
in
the
upper
limb
,
followed
by
flaccid
paralysis
in
the
affected
muscles
.
Ten
patients
had
multiple
nerve
involvement
.
Surgical
exploration
found
1
or
more
hourglass-like
constrictions
in
the
nerve
.
The
treatments
included
internal
neurolysis
,
neurorrhaphy
,
and
nerve
grafting
.
Thirty
-
one
of
42
patients
(
36
nerves
)
were
followed
up
for
a
mean
of
48
months
(
range
,
8
-
157
months
)
.
Fifteen
of
16
nerves
treated
by
neurolysis
,
10
of
13
nerves
treated
by
neurorrhaphy
,
and
4
of
7
nerves
treated
by
nerve
grafting
had
good
recovery
.
CD
8
-
positive
T
-
lymphocyte
infiltration
was
observed
in
all
the
excised
specimens
.
The
clinical
presentation
of
patients
with
hourglass-like
constrictions
in
their
nerves
is
similar
to
that
of
patients
with
neuralgic
amyotrophy
.
Histochemical
analysis
suggests
that
the
pathogenesis
may
be
immunological
in
origin
.
The
role
of
surgery
in
this
condition
is
uncertain
.
Diseases
Validation
Diseases presenting
"paralysis"
symptom
cadasil
dracunculiasis
kindler syndrome
locked-in syndrome
megacystis-microcolon-intestinal hypoperistalsis syndrome
neuralgic amyotrophy
pyruvate dehydrogenase deficiency
thoracic outlet syndrome
waldenström macroglobulinemia
This symptom has already been validated