0:01

This is an MRI of a patient

0:02

with neurofibromatosis type 1,

0:04

who's four years old.

0:05

And we can see a subtle area of myelin vacuolization

0:08

in the left globus pallidus.

0:09

Otherwise,

0:10

everything on this image looks fairly normal.

0:13

Now, I will point out in the Sylvian fissure,

0:17

the flow voids of the M2 and M3 segments

0:20

of the left middle cerebral artery,

0:22

as well as the right middle cerebral artery.

0:24

If we look at this study performed

0:28

two years later,

0:29

we can see that there's asymmetrically

0:33

decreased visualization of flow voids

0:35

in the right Sylvian fissure.

0:38

And another year later,

0:40

while we're seeing evolving areas

0:42

of myelin vacuolization,

0:43

we again see absence of significant flow voids

0:47

in the right Sylvian fissure.

0:50

Now,

0:51

one thing also to look at is FLAIR imaging.

0:56

Here is the FLAIR imaging

0:57

on the initial study,

0:59

then on follow-up,

1:02

and then the most recent study.

1:06

And look what starts to occur,

1:09

we start to see FLAIR hyperintense signal

1:12

within these vessels of the M2 and M3

1:15

branches of the right middle cerebral artery.

1:17

That is a sign of slow flow.

1:20

That is a sign of often of some

1:22

proximal stenosis or injury.

1:25

And this hypertense signal on FLAIR,

1:28

because these vessels are sort of creeping and

1:30

crawling up along the surface of the brain,

1:33

has been referred to as the ivy sign.

1:35

That is a sign that can be seen, as I said,

1:38

with proximal stenosis, in particular,

1:41

moyamoya vasculopathy.

1:43

MR angiogram confirmed.

1:46

We see the left carotid terminus.

1:49

We see the left middle cerebral artery,

1:52

the M1, M2, and M3 branches.

1:54

We're not seeing the M1, 2,

1:57

or 3 branches of the right middle

1:59

cerebral artery very well.

2:00

So, this confirms that this patient has

2:03

right-sided moyamoya vasculopathy,

2:06

which is a known association in

2:08

neurofibromatosis type 1.

2:10

Now, we don't know exactly why,

2:13

but I will say that neurofibromatosis type 1

2:15

is related to a gene defect on chromosome 17.

2:19

There is a familial moyamoya syndrome that is

2:23

also known to have a gene defect

2:26

elsewhere on chromosome 17.

2:27

So, whether it is directly related to moyamoya

2:32

vasculopathy from NF 1, or whether it is a

2:36

colocation being close on chromosome 17,

2:39

it's hard to say.

2:40

We know there are other vasculopathies in

2:43

neurofibromatosis type 1.

2:45

So either way,

2:47

it's important to recognize these patients

2:49

with neurofibromatosis type 1

2:51

can develop moyamoya vasculopathy,

2:54

and the findings can be subtle.

2:57

I will also show you

2:59

on the study where it was eventually identified.

3:01

There's also enhancement along the left meninges

3:07

and along the pia

3:08

of the right cerebral hemisphere,

3:10

likely related to collateral formation.

3:13

So, this is a patient with moyamoya

3:17

vasculopathy that was subsequently

3:18

identified prior to a stroke.

3:21

The patient was able to undergo

3:23

revascularization procedure

3:24

and has not had a stroke.

3:26

So, these are the types of findings that we can

3:29

provide a value-added service

3:30

if we can identify.