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Clinically Isolated Syndrome

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So there's a new CIS in town.

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Normally we think of CIS as carcinoma in situ,

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but for the neurologists,

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CIS refers to clinically isolated syndrome.

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This is a monophasic presentation with a

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suspicion of an inflammatory

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demyelinating disorder.

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Symptoms are typically of rapid onset

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and last for more than 24 hours.

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So this is the first event, if you will,

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in someone who potentially will have

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multiple sclerosis down the road.

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But this is a solitary event and that's why

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it's called a clinically isolated syndrome.

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Nonetheless,

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these patients with clinically isolated

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syndrome are often imaged because of this new

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neurologic event and get an MRI scan.

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The impact of MRI is very important because of

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those patients who have an abnormal MRI,

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82% will convert to multiple sclerosis

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at some point in the next 20 years.

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If, on the other hand,

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they have a single neurologic

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event with a normal MRI,

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only 21% will develop the diagnosis

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of multiple sclerosis.

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And these are different authors showing that

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the ranges can be 82% to 88%, 19% to 21%.

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But by and large,

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we say that four fifths or more of them with

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an abnormal MRI will convert to multiple

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sclerosis over the course of time.

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If the MRI shows that there is a

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gadolinium enhancing lesion,

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then you have an even higher rate at which

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these patients will convert to multiple

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sclerosis from clinically isolated syndrome.

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In addition, when they convert,

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they have greater disability.

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So this is CIS and you will see on

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your request know CIS evaluation.

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It is the pre-multiple sclerosis evaluation

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hasn't yet fulfilled the clinical criteria of

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multiple episodes of neurologic events,

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but they are suspicious of it and therefore

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looking at the MRI to help guide them.

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In some cases,

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it may help guide therapy because the patients

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may be put on immunosuppressives

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based on the MRI result.

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Now we have CIS, but we also have RIS.

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This is one of my favorite diagnoses.

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These are patients who were not admitted

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with a new neurologic event,

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but have a radiology imaging pattern that

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looks just like multiple sclerosis.

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So these are patients that may be incidentally

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discovered being evaluated, for example,

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for migraine headaches. And lo and behold,

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you find white matter lesions.

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That look just like multiple sclerosis in

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the periventricular and juxtacortical or

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infratentorial location and or enhancing lesions

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in the white matter and non-enhancing lesions.

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But the neurologists and the clinicians have

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no clinical history of a neurologic event.

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This is what is known as radiologically

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isolated syndrome, or RIS.

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8.8% of healthy relatives of patients with

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multiple sclerosis and 4.9% of non-familial

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healthy control subjects may show

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multiple white matter lesions,

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and they may indeed fulfill McDonald criteria

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for multiple sclerosis and yet

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not carry that diagnosis.

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If the patient has enhancing lesions,

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the progression to multiple sclerosis

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increases dramatically.

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So we say if you show an RIS pattern that

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is a multiple sclerosis-like pattern,

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and yet you don't have MS in your diagnosis,

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there's a one-third chance over the course of

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the next three years that you will indeed

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develop multiple sclerosis as defined by the

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clinical criteria, not just imaging criteria.

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If you have enhancing lesions,

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that rate goes up three to fourfold.

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And if you follow these patients,

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even if they don't have neurologic

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symptoms currently,

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they may show progressive MR findings in that

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they may have new MR lesions

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and new enhancing lesions,

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even though they may be asymptomatic

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neurologically.

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So this is radiologically isolated syndrome.

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In fact,

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I wrote a paper with one of my outstanding

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research fellows, Gina Pakpur.

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What we did was we looked at follow-up of

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emergency department MRI scans in patients

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who did not have a diagnosis

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of multiple sclerosis,

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but for whom we saw a pattern

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that looked just like MS.

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And what we found was if we in our reports

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said that this looks like MS, period blank.

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In other words,

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a demyelinating disorder was placed as

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the only differential diagnosis.

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These patients ultimately had a final

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diagnosis of multiple sclerosis

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over the course of time,

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and this was a six-month follow-up.

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In 84.3%,

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if MS was listed as the first in the

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differential diagnosis of a list

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of differential diagnoses,

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then 37.5% of them over the course of the next

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six months had the diagnosis of multiple

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sclerosis made. If it was in the middle,

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like the second or the third or the fourth

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or the fifth th, then as you can see,

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those rates went down precipitously.

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So the point here being that these are

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patients who show from an emergency room MR

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scan not performed for demyelinating

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disorder or even neurologic events.

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If we say this looks like MS,

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i.e.,

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radiologically isolated syndrome,

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and we say this is MS,

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it will be MS in 84.3% as a diagnosis

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within six months.

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If you look out even further

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over the course of time,

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I imagine that this would be even higher.

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But our look back was a six-month look back.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Neuroradiology

MRI

Idiopathic

Brain

Acquired/Developmental

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