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Neuromyelitis Optica Spectrum Disorder

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Another of the causes of optic neuritis,

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besides multiple sclerosis and viral infectious inflammatory

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etiologies, is the neuromyelitis optica syndrome.

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In this case, we have a patient who had left-sided visual loss.

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As we scroll the images,

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we again see abnormal signal intensity

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in the left optic nerve.

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This is seen well on the T2-weighted scan with bright

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signal intensity in the optic nerve, and we notice that

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the optic nerve sheath complex is not enlarged.

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It is, however, showing contrast enhancement.

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As we look at the remainder of the study of the brain,

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I want to highlight one aspect on the post-gadolinium

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axial scan of the orbit.

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So, I'm going to bring down

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the image of the magnified orbit,

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post-gadolinium fat-suppressed image.

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Something is different about this case than the previous

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cases of optic neuritis that I showed, and that is that

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this is a relatively large lesion

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shown here on the post-gadolinium

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axial scan, extending over a larger dimension of the

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optic nerve compared with the previous cases.

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Let's just scroll and verify that.

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You see that it extends almost to the optic canal.

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A long segment optic nerve lesion is more typical

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of the neuromyelitis optica spectrum disorder.

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Just as in the spine,

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longitudinally extensive transverse myelitis,

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where the lesions in the spine are

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extensive in superior-inferior dimension.

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It's more common in the neuromyelitis optica spectrum

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disorder than in multiple sclerosis.

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We say, in multiple sclerosis,

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the lesions in the spine are usually one to two

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vertebral segments, whereas for neuromyelias optica

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spectrum disorder, or what we're going to call NMO,

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it is usually over three segments long.

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One of the other distinguishing features about

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neuromyelitis optica spectrum disorder is the

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appearance of the lesions in the brain.

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Most of the lesions in the brain with

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NMO occur in the posterior fossa.

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As I said previously,

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it's better to look at the posterior fossa structures

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with the T2-weighted scan, rather than with a FLAIR scan

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because demyelination is better seen on the T2-weighted scan.

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Unfortunately, in the posterior fossa,

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you have the potential for phase ghosting artifacts.

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In this case,

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we see that the patient has a white matter lesion,

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which is seen in the edge of the medulla,

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near the inferior fourth ventricle.

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This white matter lesion is in a typical location for

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neuromyelitis optica, which is the area postrema.

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This is a portion of the brainstem that seems to have

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correlation with the sense of nausea and vomiting.

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The other area where NMO affects,

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but multiple sclerosis generally does not affect,

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is along the region of the hypothalamus.

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So as we scroll more superiorly,

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where we would be looking for, is lesions in this area.

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And you see that there are a few little dots of abnormal

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signal intensity in the region of the hypothalamus,

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which is typical of NMO.

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This is again a subtle finding,

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but one that you want to look for,

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particularly if one uses high-resolution imaging

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of the brainstem and the hypothalamus

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on T2-weighted imaging. Given these findings,

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we would next want to scan the spine.

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Now, initially,

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NMO was thought to be a monophasic disorder, in which the

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optic neuritis occurred at the same time as the spinal lesions.

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That has since been discredited.

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And therefore, we do recognize NMO as

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a polyphasic, multiphasic disorder.

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In which case, the optic neuritis and the transverse

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myelitis need not be concurrent.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Spine

Orbit

Non-infectious Inflammatory

Neuroradiology

Neuro

Musculoskeletal (MSK)

MRI

Head and Neck

Brain

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