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Neurologic involvement in a child with systemic capillary leak syndrome.
[systemic capillary leak syndrome]
Idiopathic
systemic
capillary
leak
syndrome
(
SCLS
)
is
a
rare
and
life-threatening
disorder
of
unknown
pathology
.
It
is
characterized
by
recurrent
episodes
of
shock
resulting
from
leakage
of
plasma
,
which
is
reflected
by
accompanying
hemoconcentration
,
hypoalbuminemia
,
and
edema
.
Since
its
first
description
(
Clarkson
B
,
Thompson
D
,
Horwith
M
,
Luckey
A
.
Am
J
Med
.
1960
;
29
:
193
-
216
)
,
there
have
been
only
3
descriptions
of
children
with
the
disorder
.
Familial
cases
have
not
been
reported
.
Brain
involvement
has
only
been
described
for
adults
and
with
minimal
radiologic
findings
.
We
report
here
an
unusual
case
of
an
8
-
year
-old
boy
with
multiple
episodes
of
SCLS
since
the
age
of
5
months
and
an
exceptional
presentation
characterized
by
substantial
neurologic
involvement
with
cerebellar
edema
and
autonomic
dysfunction
.
The
patient
's
family
history
was
remarkable
for
8
more
relatives
with
the
disorder
,
including
his
sister
who
died
during
a
similar
episode
in
infancy
and
a
first
-degree
cousin
of
his
father
who
was
diagnosed
as
suffering
from
recurrent
episodes
of
SCLS
.
Our
patient
is
,
to
our
knowledge
,
the
first
patient
with
SCLS
with
a
family
history
of
the
disorder
.
Additional
genetic
studies
in
the
extended
family
might
shed
light
on
the
pathogenesis
of
this
rare
disorder
.
Diseases
Validation
Diseases presenting
"which is reflected by accompanying hemoconcentration"
symptom
systemic capillary leak syndrome
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