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Erdheim-Chester disease with isolated craniocerebral involvement.
[erdheim-chester disease]
Erdheim-
Chester
disease
(
ECD
)
is
a
rare
non-
Langerhans
form
of
histiocytosis
with
distinctive
radiographic
and
pathological
features
.
Intracranial
involvement
is
further
a
rarity
,
usually
producing
diabetes
insipidus
or
cerebellar
-brainstem
symptoms
.
We
report
a
40
-
year
-old
man
presenting
with
recurrent
secondarily
generalised
seizures
.
An
MRI
scan
of
the
brain
revealed
multiple
enhancing
intracranial
masses
in
frontal
,
temporal
and
parietal
regions
.
Biopsy
from
the
left
frontotemporal
lesion
confirmed
it
to
be
a
rare
case
of
ECD
.
The
patient
received
a
short
course
of
corticosteroids
initially
and
subsequently
remained
well-controlled
on
antiepileptic
therapy
alone
.
A
repeat
MRI
of
his
brain
showed
significant
resolution
of
lesions
.
Osteolytic
lesions
in
the
skull
vault
were
detected
during
follow-up
which
also
disappeared
.
Interestingly
,
there
was
no
involvement
of
long
bones
or
any
other
system
even
after
12
years
of
follow-up
.
Diseases
Validation
Diseases presenting
"long bones"
symptom
achondroplasia
cohen syndrome
dentin dysplasia
erdheim-chester disease
pyomyositis
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