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Optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease).
[malignant atrophic papulosis]
A
fatal
case
of
malignant
atrophic
papulosis
(
Degos
disease
)
with
optic
nerve
and
spinal
cord
involvement
is
described
.
Magnetic
resonance
imaging
(
MRI
)
of
the
optic
nerve
showed
abnormal
signal
enhancement
on
fat
suppressed
T
1
weighted
images
after
intravenous
meglumine
gadopentetate
infusion
.
On
T
2
weighted
sagittal
images
,
a
sawtooth
pattern
was
observed
over
seven
vertebral
segments
of
the
spinal
cord
.
On
necropsy
,
a
severe
loss
of
myelinated
nerve
fibres
in
the
left
optic
nerve
was
seen
,
with
thrombotic
obstruction
of
the
central
retinal
artery
.
Spongy
degeneration
was
observed
in
all
levels
of
the
spinal
cord
,
with
patchy
and
motheaten
patterns
caused
by
thromboses
and
endothelial
proliferation
in
subarachnoid
vessels
.
Findings
on
MRI
were
consistent
with
findings
on
pathological
examination
.
Diseases
Validation
Diseases presenting
"with patchy and motheaten patterns caused by thromboses and endothelial proliferation in subarachnoid vessels"
symptom
malignant atrophic papulosis
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