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Neurofibromatosis Type 2

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As we discuss schwannomas of the spinal canal,

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I do want to refresh your memory about the

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entity of neurofibromatosis type 2.

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Neurofibromatosis type 2 is also known as the

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MISME syndrome. And let s make sure it's MISME ,

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not miss me. We don't want to miss this diagnosis.

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MISME stands for Multiple Inherited Schwanomas,

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Meningiomas and Ependymomas.

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So in neurofibromatosis type 2,

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you actually may find three lesions in the spinal

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canal. You can find the schwannomas,

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you can find the meningiomas,

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and you can have intramedullary ependymomas.

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The majority of the lesions are

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going to be schwannomas.

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And you'll notice that the MISME syndrome or

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neurofibromatosis type 2, remember,

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is associated with the bilateral

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vestibular schwannomas.

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Here is a patient with neurofibromatosis type 2.

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We have post-gadolineum-enhanced scans in the

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cervical and thoracic region, as well as the lumbar

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region. When we look in the lumbar region,

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we can see that the patient has multiple enhancing

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lesions associated with the

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cauda equina nerve roots.

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So these are all, on this post-gad T1-weighted scan,

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multiple schwannomas that are in the lumbar

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plexus of the cauda equina nerve roots.

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However, if we look carefully at the image to the left,

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we also see a lesion that is enhancing within the

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spinal cord. And that would be your ependymoma.

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We see all these little schwannomas on the surface of

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the spinal cord in the intradural

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extramedullary space,

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but we do have the ependymoma in the

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intradural intramedullary space,

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which is all part of neurofibromatosis type 2.

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Here's another patient with neurofibromatosis type 2.

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And we are looking on serial axial scans,

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and what do we see?

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We see these large

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extradural schwannomas, as well as some that are in

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the soft tissues of the back and the muscle.

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We see that there is a lesion here which is

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affecting the intradural extramedullary compartment,

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pushing the spinal cord anteriorly

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into the left side.

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These would be schwannomas in the intradural

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extramedullary compartment.

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And we also see a lesion which is centered

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within the spinal cord.

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And that would be our intradural

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intramedullary ependymoma.

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The cauda equina nerve roots are enhancing a little

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bit more than one would expect and that is included

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in our multiple schwannoma inherited syndrome

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of neurofibromatosis type 2.

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You'll notice that the patient also has

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an intramuscular schwannoma. Again,

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extradural, intradural, extramedullary, as well as

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intradural intramedullary lesions of neurofibromatosis

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type 2. And here's another example,

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same with multiple intramedullary lesions.

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Multiple ependymomas in the spinal cord seen in the

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cervical region in addition to multiple small

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schwannomas in the cauda equina nerve roots seen

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on the right hand image down low.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Spine

Neuroradiology

Neoplastic

Musculoskeletal (MSK)

MRI

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