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Sylvian aqueduct syndrome with slit ventricles in shunted hydrocephalus due to adult aqueduct stenosis.
[hydrocephalus with stenosis of the aqueduct of sylvius]
The
authors
report
on
3
patients
who
developed
sylvian
aqueduct
syndrome
(
SAS
)
in
the
context
of
shunt
dysfunction
and
slit
ventricles
.
All
3
patients
had
received
shunts
for
adult
onset
hydrocephalus
due
to
aqueduct
stenosis
and
were
stable
for
years
before
presenting
with
loss
of
upward
gaze
,
convergence-retraction
nystagmus
,
and
slit
ventricles
,
all
due
to
shunt
overdrainage
.
All
3
improved
after
either
shunt
revision
or
a
third
ventriculostomy
procedure
.
Although
it
is
well
known
that
SAS
can
be
caused
by
shunt
blockage
producing
a
transtentorial
pressure
gradient
,
these
cases
emphasize
that
an
identical
clinical
pattern
can
occur
with
a
reverse
transtentorial
pressure
gradient
and
slit
ventricles
due
to
shunt
overdrainage
.
The
authors
propose
a
simple
management
plan
for
patients
with
shunted
hydrocephalus
who
develop
SAS
.
Diseases
Validation
Diseases presenting
"nystagmus"
symptom
aniridia
gm1 gangliosidosis
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
kabuki syndrome
oculocutaneous albinism
primary hyperoxaluria type 1
well-differentiated liposarcoma
wolf-hirschhorn syndrome
zellweger syndrome
This symptom has already been validated