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Wk 1, Case 1 - Review

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Yeah, so the first case was an example of, um, a a,

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a patient that had abnormal enhancement involving the optic nerve and

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the optic nerve sheath on the left. And then when we look at the, uh,

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T two weighted images, we can see these areas of,

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let me window a little better here.

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We can see these areas of, uh,

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multiple white matter hyperintensities that are actually seen throughout the

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brain parenchyma.

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And this lesion right here kind of tips you off that we're probably dealing

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with, um,

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multiple sclerosis as opposed to metastases just because you have this sort of

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rim of enhancement. And in a way it's this little,

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sort of this thick and flare sequence too.

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And then when you look at this lesion right here,

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you can see it's sort of extending along the white matter tracks.

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So when we look at the contrast enhanced study, uh,

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we can see, uh, multiple lesions that are enhancing. Um,

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and I think if someone did say lepto menal metastases,

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I don't think that's necessarily, um, wrong. I think it's a good thought,

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but sort of given the age of the patient and also given the orientations,

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I think, um, uh, MS really is, is the best option.

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So what I wanted to do, um, just really briefly is just give just a,

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a couple of slides on optic neuritis.

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So for the optic nerve itself, I know the classic thing,

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and I kind of grew up with this as well too,

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is that when we're looking at lesions that are involving the orbit,

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we're always sort of trained to look at intracon versus extra conal. Um,

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and to be honest with you,

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the reason this kind of all developed is from the days of ct.

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So unfortunately,

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I grew up in the days of CT and really before we were doing m r,

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the orbits and the most consistent anatomic structures that we could see in the

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orbit were actually the extraocular muscle.

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So we could see the superior rectus muscle.

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You can see a little bit of the lator, paler muscle, medial rectus,

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lateral rectus, inferior rectus. And these muscles actually,

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um, form the cone of the orbit, if you will.

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And that's how we sort of created this differential diagnosis of intracon versus

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extra cline. I think that's actually, you know, taught, you know, for me,

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what I've always liked to do is, um,

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I think it's a little bit rudimentary just to look at the extraocular muscles

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alone. So when you really do good imaging,

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you should be able to see the optic nerve.

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So this area surrounding is the dural sheath.

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Then we have this area of increased T two signal, which is the C S F space.

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And then right in the middle of that nerve, uh, that complex is the optic nerve.

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So you have the optic nerve, the C s F, and then you have this dural sheet.

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So for me,

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I like to give my differential diagnosis based on the relationship of the optic

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nerve as opposed to intracon versus extra conal because as you'll see

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in this first week, um, there are a lot of masses that can

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Occur in the orbit, but some of these, um,

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are if can involve either within the cone or outside of the cone

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and it can make things confusing. So I like to,

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to give my differential diagnosis based on the optic nerve.

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So this is what I was referring to today in the, in the old days, and this,

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this was actually a very old study when I was actually a fellow down in,

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in Gainesville, um, back in the last century. Um,

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just nicely demonstrates that yeah, we can see the optic nerve,

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but the most reliable features 30 years ago was actually the muscle cone.

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So that's how it started. But moving forward again, we should be able to,

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to base it on the optic nerve.

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So this is an example of optic neuritis.

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This is diffuse enhancement here involving the, the right optic nerve.

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And then when we look at the coronal images,

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we can see this very robust enhancement involving the nerve.

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And you can see it on the opposite side. So when you do,

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you do your intracranial versus extraoral differential diagnosis, yes,

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optic neuritis is quote unquote in the differential for intracon lesions.

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Now, if you, if, if you understand the anatomy of the optic nerve,

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then you can actually begin to grade optic neuritis based on

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what we see on good quality imaging.

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So this is an example of unilateral optic neuritis and we can see the abnormal

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enhancement of the nerve sheath.

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And then when we look at the coronal T two weighted images with fat suppression,

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we can see a little bit of increased signal here involving the nerve.

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And if you look real closely,

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you can actually see asymmetrical increased signal surrounding the C

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S F.

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And that really is a consistent finding when you have cases of optic neuritis.

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In fact, um, at times when we look,

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and I'll go over these in just a in a second,

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when we start looking at the subacute and the chronic forms,

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that asymmetrical signal in that C S F is actually a pretty reliable signal.

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So in acute optic neuritis,

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the main finding is really gonna be abnormal enhancement.

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And then you may start to see some increased signal in the sheath.

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Now what happens in subacute optic neuritis is that that signal goes away,

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the enhancement goes away. But when you look at the T two weighted images,

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look at this,

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you can see the signal within the optic nerve is higher signal on the involved

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side. And then you can see the high T two signal surrounding that optic nerve.

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And to be honest with you, you know,

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I would say the majority of cases of optic neuritis that I see are probably

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somewhere in the peri subacute phase.

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And occasionally by the time the patients develop this vision loss and they

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think they have optic neuritis by the time they present to the ophthalmologist

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or the optometrist and then they get their imaging study done,

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sometimes that enhancement is completely gone away or it'll just see a little

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bit. So to be honest with you,

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I really take a really hard look at the coronal T two weighted images, um,

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to see if I see those associated findings.

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And then eventually in chronic optic neuritis,

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What happens is that you can see that optic nerve right here and then eventually

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it shrinks and it shrinks to almost like a pinpoint nerve. And then again,

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look at the high signal surrounding the optic nerve on the right side compared

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to the left side. So when you get really good in optic neuritis,

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not only can you make the diagnosis,

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but you can actually grade it based on the enhancement pattern and also the

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caliber of the optic nerve and the surrounding T two signal. Now,

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what has been described, um,

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as we've learned more about optic neuritis and,

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and actually about multiple sclerosis,

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is that there sort different types and these are really based on specific as you

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know, biomarkers, um, basically liquid biopsies if you will.

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So the classic multiple sclerosis can give you this unilateral optic neuritis.

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So this is just a classic case of left optic neuritis.

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Now this was something that we used to call devex disease.

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This is neuromyelitis optica and it's been identified to be associated with anti

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aquaporin four. And so in dev X disease,

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this is the classic thing about optic neuritis associated with abnormal signal

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within the cervical cord. So you'd get these plaques in the cervical cord.

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So in dev X disease, and I should say N M O is the,

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is really the proper term. This, uh,

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enhancement pattern has a tendency to be bilateral.

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So you can see it bilateral here.

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And this is more associated with aquaporin four.

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And then this is something that's been described really has gotten a lot of

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press over the last five to seven years,

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is that now you can identify a form of a demyelinating process

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that can result in,

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in a type of optic neuritis that's called myelin oligodendrocyte,

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oligodendrocyte glycoprotein or mog.

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And M o G has a tendency to involve the anterior portion of the optic nerves and

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it tends to be more severe. Now, uh, you know,

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a few years ago when we started figuring out that there were different types of

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optic neuritis, there was some thought that hey, maybe we could actually,

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you know, identify the type of optic neuritis based on the enhancement pattern.

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And it doesn't always work. But in general,

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and this is really a rough rule,

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if you see unilateral involvement of an optic nerve that's more consistent with

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multiple sclerosis,

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if you see bilateral involvement and especially involvement to the posterior

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portion of the nerve as is seen here,

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then that is a little bit more consistent with N M O.

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And then if you see really robust enhancement involving the anterior half of the

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optic nerves, especially with extending into the nerve globe junction,

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that's more suggestive of M O G because these patients tend to have a, a,

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a more robust form of optic neuritis, a more severe form. But again,

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those are more rules of thumb than, than really hard rules.

Report

Patient History

Papilledema

Findings
Pre and post contrast MR was performed of the brain and the orbits.

Brain: Sagittal T1-weighted images demonstrate corpus callosum to be intact. No evidence of Chiari malformation. No abnormal pineal region masses. Mild flattening of pituitary gland. Diffusion imaging demonstrates no evidence of recent infarct. Contrast-enhanced T1-weighted images demonstrates multiple focal enhancing intraaxial lesions seen throughout the brain parenchyma predominantly involving the juxtacortical, centrum semiovale, periventricular white matter and periatrial regions. The most dominant area of enhancement is a 1.6cm x 1.1cm ring-enhancing lesion involving the left centrum semiovale. These areas of enhancement are associated with multiple ill-defined areas of increased FLAIR and T2 signal and most consistent with active multiple sclerosis.

Orbits: There is diffuse long segment enhancement of the left retrobulbar optic nerve which extends posterior to the orbital apex. The enhancement involving the optic nerve is predominately involving the dural sheath. This is somewhat atypical for optic neuritis as the enhancement is typically involving the optic nerve. However, it is possible this could be due to acute and extensive nature of this disease. No definite evidence of enhancement is identified involving the right retrobulbar optic nerve. No abnormal intraocular enhancing lesions are identified.

No abnormal sellar or juxtasellar enhancing lesions are seen. No evidence of compressive lesions are identified involving the optic chiasm. No abnormal lesions are identified involving the anterior skull base or olfactory sulcus. The bilateral gyrus rectus are normal. No evidence of aggressive skull base lesions are seen. No abnormal enhancing lesions are identified in the region of the perimesencephalic cistern. The region of Meckel's cave is symmetric.

Conclusions
1. Multiple intraparenchymal enhancing lesions associated with numerous white matter abnormalities and enhancement of the retrobulbar left optic nerve. The findings are consistent with acute multiple sclerosis and left optic neuritis. The peripheral enhancement of the left optic nerve is somewhat atypical for acute optic neuritis. However, this could be due to the acute nature of the disease. These findings should be correlated with the clinical symptoms and other diagnostic features of multiple sclerosis.

2. No evidence of ventriculomegaly.

Case Discussion

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Suresh K Mukherji, MD, FACR, MBA

Clinical Professor, University of Illinois & Rutgers University. Faculty, Michigan State University. Director Head & Neck Radiology, ProScan Imaging

Tags

Neuroradiology

MRI

Head and Neck