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Intracranial Arteriopathy in Tuberous Sclerosis Complex.
[lymphangioleiomyomatosis]
Arterial
aneurysms
,
mostly
aortic
and
intracranial
,
have
been
occasionally
reported
in
patients
with
tuberous
sclerosis
complex
.
Brain
magnetic
resonance
imaging
reports
of
404
patients
with
definite
and
16
patients
with
either
probable
or
possible
tuberous
sclerosis
complex
were
revised
for
intracranial
aneurysms
.
Among
these
patients
,
brain
images
of
220
patients
with
definite
and
16
with
probable
or
possible
tuberous
sclerosis
complex
were
reviewed
.
Intracranial
aneurysms
were
reported
in
3
of
404
patients
with
a
definite
diagnosis
(
0
.
74
%
)
(
general
population
:
0
.
35
%
)
,
including
2
children
.
A
fourth
intracranial
aneurysm
was
found
in
a
patient
with
probable
tuberous
sclerosis
complex
,
who
did
not
have
tubers
or
subependymal
nodules
but
had
clinical
manifestations
related
to
neural
crest
derivatives
,
including
lymphangioleiomyomatosis
and
extrarenal
angiomyolipomas
.
The
authors
hypothesize
that
neural
crest
dysfunction
can
have
a
major
role
in
intracranial
arteriopathy
in
tuberous
sclerosis
complex
,
as
smooth
muscle
cells
in
the
forebrain
vessels
are
of
neural
crest
origin
.
Diseases
Validation
Diseases presenting
"mostly aortic and intracranial"
symptom
lymphangioleiomyomatosis
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