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Prepare trainees to be on call for the emergency department with this specialized training series.
22 topics, 1 hr. 12 min.
Introduction to Neurocutaneous Syndromes Part 1 (NF1)
1 m.Chiari Malformation Type 1 in a Patient with NF1
3 m.NF1, With Waxing and Waning Cystic Lesion
5 m.NF1 with Developing Myelin Vacuolization, and Optic Glioma
4 m.NF1 with Suspicious Lesions and Tortuous Optic Nerve
7 m.NF1 with Bilateral Thalamic Lesions and Differential Diagnosis
5 m.Normal Brain MRI in Patients with NF1
5 m.Mild NF1 Phenotype with Sphenoid Wing Dysplasia
4 m.NF1 with High Grade Glioma
3 m.NF1 with Bilateral Optic Nerve Glioma
3 m.Focal Optic Pathway Glioma in NF1
4 m.NF1 with Fusiform Optic Pathway Glioma
3 m.NF1 with Optic Chiasmatic Glioma
3 m.NF1 with Evolution of Optic Nerve Glioma
5 m.NF1 with Optic Pathway Glioma and Moyamoya Disease
6 m.Developing Right Fusiform Optic Glioma
3 m.Glaucoma, an Orbital Manifestation of NF1
3 m.NF1 with a Brain Stem Lesion and Optic Nerve Glioma
4 m.NF1 with Buphthalmos and Orbital Plexiform Neurofibroma
4 m.MRI Appearance of Sphenoid Wing Dysplasia in NF1
2 m.NF1 with Moyamoya Vasculopathy
4 m.Neurocutaneous Syndromes Part 1 (NF1) Summary
2 m.0:01
This is an MRI of the brain
0:02
in an eight-year-old child
0:03
with neurofibromatosis type 1.
0:05
We see areas of myelin vacuolization
0:07
in the globus pallidus bilaterally and
0:08
in the left cerebral peduncle,
0:11
in the left ventral pons,
0:13
in the region of the dentate nucleus,
0:15
and deep cerebellar gray and white matter
0:17
bilaterally, left greater than right.
0:19
We can see, actually,
0:21
an expansile area in the left posterior
0:23
aspect of the medulla oblongata
0:28
Now, we don't see any abnormal enhancement in
0:30
that area of expansile involvement
0:33
in the medulla oblongata,
0:35
but it's definitely something to
0:36
be aware of and keep an eye on.
0:39
But the most important thing to notice
0:43
in this patient is this focal lesion here.
0:47
This is in the parasagittal aspect
0:48
of the left frontal lobe.
0:51
Heterogeneously hyperintense
0:53
on T2-weighted imaging with areas of actual
0:56
relative T2 hypointense signal.
0:59
It's relatively homogeneously hypointense
1:02
on T1 weighted imaging
1:06
and demonstrates heterogeneous
1:07
post-contrast enhancement.
1:12
If I look on diffusion-weighted imaging,
1:13
I see hyperintense signal
1:16
that the ADC maps.
1:20
The ADC maps confirm as being relatively
1:22
reduced water diffusion with
1:24
an average ADC of 940.
1:28
Now, generally speaking,
1:29
while not an exact cutoff below 1000 for
1:33
the ADC is often associated
1:36
with high-grade gliomas.
1:40
So, this patient with neurofibromatosis type 1
1:43
has this focal lesion with
1:46
surrounding mass effect and edema,
1:48
heterogeneous enhancement,
1:50
and reduced water diffusion.
1:52
At biopsy,
1:54
this was confirmed to be a high-grade glioma.
1:56
So this is the patient with
1:58
neurofibromatosis type 1,
1:59
who developed a high-grade glioma.
Interactive Transcript
0:01
This is an MRI of the brain
0:02
in an eight-year-old child
0:03
with neurofibromatosis type 1.
0:05
We see areas of myelin vacuolization
0:07
in the globus pallidus bilaterally and
0:08
in the left cerebral peduncle,
0:11
in the left ventral pons,
0:13
in the region of the dentate nucleus,
0:15
and deep cerebellar gray and white matter
0:17
bilaterally, left greater than right.
0:19
We can see, actually,
0:21
an expansile area in the left posterior
0:23
aspect of the medulla oblongata
0:28
Now, we don't see any abnormal enhancement in
0:30
that area of expansile involvement
0:33
in the medulla oblongata,
0:35
but it's definitely something to
0:36
be aware of and keep an eye on.
0:39
But the most important thing to notice
0:43
in this patient is this focal lesion here.
0:47
This is in the parasagittal aspect
0:48
of the left frontal lobe.
0:51
Heterogeneously hyperintense
0:53
on T2-weighted imaging with areas of actual
0:56
relative T2 hypointense signal.
0:59
It's relatively homogeneously hypointense
1:02
on T1 weighted imaging
1:06
and demonstrates heterogeneous
1:07
post-contrast enhancement.
1:12
If I look on diffusion-weighted imaging,
1:13
I see hyperintense signal
1:16
that the ADC maps.
1:20
The ADC maps confirm as being relatively
1:22
reduced water diffusion with
1:24
an average ADC of 940.
1:28
Now, generally speaking,
1:29
while not an exact cutoff below 1000 for
1:33
the ADC is often associated
1:36
with high-grade gliomas.
1:40
So, this patient with neurofibromatosis type 1
1:43
has this focal lesion with
1:46
surrounding mass effect and edema,
1:48
heterogeneous enhancement,
1:50
and reduced water diffusion.
1:52
At biopsy,
1:54
this was confirmed to be a high-grade glioma.
1:56
So this is the patient with
1:58
neurofibromatosis type 1,
1:59
who developed a high-grade glioma.
Report
Description
Faculty
Asim F Choudhri, MD
Chief, Pediatric Neuroradiology
Le Bonheur Children's Hospital
Tags
Syndromes
Pediatrics
Neuroradiology
Neuro
Neoplastic
MRI
Brain
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