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Clinical features and long-term follow-up of quasi-moyamoya disease in children.
[proteus syndrome]
Inherited
or
acquired
disorders
and
conditions
may
present
in
conjunction
with
moyamoya
disease
.
This
condition
is
known
as
quasi-
moyamoya
disease
.
A
retrospective
review
of
69
moyamoya
disease
patients
treated
for
the
past
20
years
identified
7
patients
with
quasi-
moyamoya
disease
and
5
of
them
were
pediatric
patients
.
The
mean
age
at
initial
diagnosis
was
6
.
4
years
(
range
5
-
9
)
.
Associated
disorders
were
:
craniosynostosis
,
dwarfism
with
coarctation
of
aorta
,
Proteus
syndrome
,
and
cranial
irradiation
for
brain
tumor
.
Their
clinical
type
included
cerebral
ischemia
in
3
patients
,
cerebral
bleeding
with
ischemia
in
1
and
epilepsy
in
1
.
The
3
patients
with
cerebral
ischemia
underwent
bypass
surgery
and
their
ischemia
was
improved
.
One
patient
died
of
brain
tumor
recurrence
and
the
activities
of
daily
living
in
the
remaining
patients
were
affected
by
mental
retardation
.
The
clinical
course
and
radiological
finding
of
quasi-
moyamoya
disease
are
diverse
because
of
associated
disorders
,
distinguishing
definite
moyamoya
disease
.
Diseases
Validation
Diseases presenting
"dwarfism with coarctation of aorta"
symptom
proteus syndrome
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