Get a Group Membership for your Organization. Free Trial
Pricing
Free TrialLogin

Intracranial Manifestation of Tuberous Sclerosis Complex

HIDE
PrevNext

0:00

This is a FLAIR flare MR image

0:02

from a ten-month-old with

0:04

tuberous sclerosis complex.

0:05

The tuberous sclerosis complex was identified

0:08

on prenatal imaging with a cardiac rhabdomyoma,

0:12

and subsequent genetic testing confirmed it.

0:14

So, it's known that the patient has tuberous sclerosis complex.

0:17

Now, at ten months of age,

0:19

They are performing an MRI of the brain

0:20

to see what the intracranial involvement is.

0:24

So this FLAIR image,

0:26

we're not necessarily, at first glance,

0:29

not seeing anything major.

0:30

We see a little subependymal nodule here.

0:34

We see another subependymal nodule.

0:37

So as we look through it, as we look closer,

0:41

we're seeing some juxtacortical signal

0:43

abnormality in the left frontal pole,

0:47

in the left temporal lobe.

0:49

Now, we're seeing some cystic areas at the junction

0:53

of the left temporal and occipital lobes.

0:55

So, it's not normal.

0:57

So, there are intracranial manifestations

0:59

of tuberous sclerosis complex.

1:02

But note that this is,

1:04

while this is a FLAIR image,

1:05

the white matter is sort of hyperintense.

1:07

We usually aren't used to seeing

1:09

the white matter look like that on FLAIR.

1:11

That's because at this age,

1:13

at nine and ten months of age,

1:14

the brain is partially myelinated.

1:17

So, FLAIR can look very unusual.

1:20

So, if we look at T2-weighted imaging,

1:25

we can see multifocal areas

1:27

of cortical dysplasia,

1:29

much more so than we would suspect on FLAIR.

1:33

So on the FLAIR,

1:34

we see this little crescentic area of

1:36

juxtacortical signal abnormality.

1:37

But that corresponds to this area here

1:41

on the T2-weighted image.

1:42

But these multifocal additional areas on T2,

1:46

you can barely tell on FLAIR.

1:48

So, this just goes to show understanding what

1:52

imaging sequences to use at a given

1:54

age of development is important.

1:56

Now, in neonates,

2:00

where they're completely unmyelinated.

2:02

T1-weighted imaging is typically the best way

2:05

to look for areas of cortical dysplasia

2:07

in tuberous sclerosis complex.

2:10

Well here, we can see this bright signal.

2:13

That bright signal is related

2:14

to ongoing myelination.

2:16

It's hyperintense T1-weighted imaging

2:18

from the proteolipids of myelin.

2:21

And so, we're not really seeing the

2:23

T1-weighted manifestation of cortical dysplasia.

2:27

At this stage of intermediate myelination,

2:29

T1-weighted imaging underestimates

2:31

the lesion burden,

2:33

even though T1-weighted imaging is

2:35

the preferred method in a newborn.

2:38

FLAIR imaging underestimates the abnormality

2:42

in this nine to ten month age window,

2:45

given the ongoing myelination,

2:46

even though FLAIR imaging is the most commonly

2:49

used for maturely myelinated individuals.

2:52

So T2-weighted imaging itself,

2:55

without any inversion pulse,

2:56

ends up being a very effective way of

3:00

evaluating dysplasia,

3:02

especially with

3:04

a mixed ongoing myelination pattern.

3:08

Now, it also shows these subependymal nodules.

3:11

So, this is a patient who's ten months

3:14

of age with tuberous sclerosis complex,

3:17

where the T1-weighted imaging,

3:20

you typically used in a newborn,

3:23

typically used in a mature myelinated individual,

3:26

underestimate the lesion burden.

Report

Description

Faculty

Asim F Choudhri, MD

Chief, Pediatric Neuroradiology

Le Bonheur Children's Hospital

Tags

Syndromes

Pediatrics

Neuroradiology

Neuro

MRI

Brain

© 2024 MRI Online. All Rights Reserved.

Contact UsTerms of UsePrivacy Policy