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Erdheim-Chester disease of the central nervous system: new manifestations of a rare disease.
[erdheim-chester disease]
ECD
is
a
rare
non-
Langerhans-cell
histiocytosis
,
which
can
involve
the
CNS
;
therefore
,
CNS
imaging
findings
have
been
described
in
only
a
small
number
of
patients
.
To
gain
additional
insight
into
the
CNS
manifestations
of
ECD
,
we
reviewed
the
findings
on
imaging
of
the
brain
,
head
and
neck
,
and
spine
in
patients
with
ECD
who
presented
to
our
institution
.
Here
,
we
illustrate
manifestations
that
have
not
,
to
our
knowledge
,
been
previously
described
.
CT
,
MR
imaging
,
and
PET
/
CT
studies
of
the
brain
,
maxillofacial
region
,
and
spine
were
reviewed
in
11
patients
with
ECD
.
F
our
new
manifestations
of
ECD
were
present
,
including
the
following
:
a
stellate
appearance
of
intracranial
extra
-
axial
lesions
,
ependymal
enhancement
along
the
lateral
ventricle
with
deep
linear
extension
to
the
lentiform
nucleus
,
irregular
enhancement
in
the
pons
,
and
diffuse
involvement
of
the
vertebral
column
on
PET
/
CT
.
ECD
has
a
variety
of
imaging
appearances
in
the
CNS
,
including
new
manifestations
described
herein
.
Neuroradiologists
should
be
aware
of
these
manifestations
to
avoid
mistaking
them
for
other
disease
processes
.
Diseases
Validation
Diseases presenting
"ependymal enhancement along the lateral ventricle with deep linear extension to the lentiform nucleus"
symptom
erdheim-chester disease
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