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A Rosenthal fiber encephalomyelopathy resembling Alexander's disease in 3 sheep.
[alexander disease]
We
report
an
encephalomyelopathy
in
three
18
-
month
-old
Merino
sheep
with
features
of
adult-onset
Alexander
's
disease
(
AD
)
,
a
human
primary
astrocytic
disorder
.
The
signature
histologic
finding
was
the
presence
of
numerous
hypereosinophilic
,
intra-astrocytic
inclusions
(
Rosenthal
fibers
)
,
mainly
in
perivascular
,
subpial
,
and
subependymal
sites
,
especially
in
the
caudal
brain
stem
and
spinal
cord
.
Although
AD
usually
results
from
mutations
in
the
glial
fibrillary
acidic
protein
(
GFAP
)
gene
,
no
such
mutation
was
detected
in
these
sheep
.
However
,
the
annual
clinical
presentation
of
this
disorder
in
a
few
sheep
in
the
affected
flock
is
suggestive
of
a
familial
pattern
of
occurrence
.
Diseases
Validation
Diseases presenting
"especially in the caudal brain stem and spinal cord"
symptom
alexander disease
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