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[Von Hippel-Lindau syndrome].
[von hippel-lindau disease]
Von
Hippel-
Lindau
syndrome
is
an
autosomal
dominant
inherited
phacomatosis
with
a
predisposition
for
the
central
nervous
system
and
retina
.
There
is
variable
expression
with
hemangioblastomas
in
the
brain
,
medulla
oblongata
,
spinal
chord
,
renal
carcinoma
,
pheochromocytoma
,
pancreatic
cysts
and
islet
cell
tumors
as
well
as
tumors
of
the
endolymphatic
sac
of
the
inner
ear
.
Clinical
symptoms
occur
first
after
an
age
of
approximately
30
Â
years
.
Magnetic
resonance
imaging
(
MRI
)
of
the
neuroaxis
is
indicated
in
all
patients
with
a
suspicion
of
von
Hippel-
Lindau
syndrome
.
