Part II
THE MCDONALD CRITERIA
What is the Neurologist Looking For?
The actual terms used by MS Specialists in describing the diagnostic characteristics of MS are Dissemination in Space, Dissemination in Time, and the Exlcusion of Any Better Explanation for the patient's symptoms and findings.
Dissemination in Space - This means that there is evidence that the disease has attacked more than one area of the Central Nervous System. The disease has "spread out" in location.
Dissemination in Time - This means that there have been attacks on the Central Nervous System on more than one occasion, and that the subsequent attack was in at least one different spot than the first attack. The disease has spread out in time. It has been active more than once. The attacks must have occurred at least 30 days apart.
Exclusion of Better Explanations - To do this the neurologist will order many blood tests looking for MS mimics, such as causes of autoimmune vasculitis, CNS infections like Lyme Disease and syphillis, blood clotting disorders like Hughes Syndrome, deficiencies, and heavy metal poisoning. Also other tests may be done like an EEG, sleep study, EMG, and Nerve Conduction Studies.
The McDonald Criteria - 2001
In 2001, a new set of diagnostic criteria were proposed and accepted by the MS world of doctors and researchers. The McDonald criteria were very good, in that, for the first time they described criteria for the diagnosis of Primary Progressive MS, but they also allowed for the definitive diagnosis of MS in it’s earliest form (often called the Clinically Isolated Syndrome, when there had been only one “attack” or onset of one symptom. The diagnosis of these two situations requires real thought and documented abnormalities on the lab and imaging tests. These criteria were superior to ealier diagnostic guides in that they were better at picking the MS cases accurately and eliminating the non-MS cases.
For the first time, neurologists could use the information from MRIs to substitute for an attack or for more evidence of clinical lesions when the clinical history was not enough to show a pattern of the spread of the disease in time and space. This allowed many people to be diagnosed earlier. In the last 10 years, MRI has become more and more important in the diagnosis of MS. The problem is that some neurologists have come to rely solely on the MRI results and may neglect the patient's history and physical almost completely. As you examine the McDonald Criteria, you will see that the Criteria never intended that the MRI assume the first and only role.
By theMcDonald Criteria, patients fall into one of three categories: Definite MS, Possible MS and Not MS. The “type of MS” (RRMS, SPMS, etc) is determined after diagnosis and is based almost entirely on the patient's clinical course.
There were problems, though, in the practical use of the McDonald Criteria. The MRI criteria were very stringent and difficult to figure out. Also, new techniques in MRI imaging made visualizing spinal lesions easier. It was clear that spinal lesions needed a larger role in the diagnosis.
The Revised MCDONALD CRITERIA (2005)
In 2005, a group of MS specialists reconsidered the original criteria, loosened some of the MRI requirements, changed the role of the results of CSF testing and VEP in the diagnosis of PPMS, and increased the importance of spinal MRI lesions. These changes have been shown to be as good or better at picking up patients with MS and in excluding patients who do not have it.
Below is a chart ( or there will be in the Health Page for this topic) summarizing the revised criteria. After the chart is a text description in more detail about the different cases a neurologist finds.
Table: There will be a table for the Health Page that will come out of this.
DEFINITIONS
What Provides MRI Evidence of Dissemination in Space?
This is the description of a postivie MRI for the purposes of showing that there has been "dissemination in space." This would be needed if there is only evidence on neurologic exam of one clinical lesion. There is only one abnormality that points directly to a damaged area in the CNS. This is also where the misunderstanding about always needing 9 lesions on the MRI. (Sometimes you do, but not always.) In general lesions should be larger than 3mm in cross-section.
You need to have 3 of the following 4 things:
1 (one) contrast-enhancing lesion of the brain or spinal cord. If no enhancing lesion, then need 9 T2 hyperintense lesions in brain or spine.
1 infratentorial lesion or a cord lesion (this means under the tentorium, which is the membrane on which the larger cerebrum sits. Below the tentorium is the cerebellum, brainstem and spinal cord.)
1 (one) or more juxtacortical lesions (this involves nerve fibers - called U-fibers - that extend from the white matter in the subcortical area through the boundary with the cortex of the gray matter)
3 or more periventricular lesions (these are lesions sitting adjacent or very close to the ventricles)
note: Individual cord lesions can substitute along with individual brain lesions to reach the required number of T2 lesions.
What Provides MRI Evidence of Dissemination in Time?
A Contrast-Enhancing (therefore new) MRI lesion seen in a scan done at least 3 months after the onset of the first attack and at a site that is different from the site of the inital clinical attack. (an example would be: first attack was with Optic Neuritis. 3 months later there is an enhancing lesion in the frontal lobe)
OR
A new T2 lesion detected in a scan done at any time compared to an earlier scan. The earlier scan must have been done at least 30 days after the beginnig of the first clinical attack.
What is a Positive CSF (Spinal fluid) Result?
Two or more unique oligoclonal bands found in the CSF, but not in the serum OR an elevated IgG Index
What is a Positive VEP?
A delayed optic nerve signal (usually longer than 115msec), but a well-preserved wave form
A text description of the Criteria in action is next.
Quix
Part III
THE MCDONALD CRITERIA 2005
What Does the Chart Say in Real Words?
The neurologist is usually faced with one of several situations. Each one begins with an analysis of the patients history of symptoms looking for a pattern of attacks and a thorough neurologic exam to look for clinical lesions. Ideally the patient would have seen a physician for each of these attack, but this often is not the case. Then the picture becomes much murkier. Each scenario below assumes that all other reasonable expalnations for the patient's problems has been ruled out. They are listed by ease of making the diagnosis.
First scenario
2 attacks
2 clinical lesions
The patient has had 2 or more attacks by history, with a clearcut remission between at least two of them. A doctor has found neurologic abnormalities during at least 2 attacks. There are or have been at least two objective clinical lesions in separate parts of the body. In order to make the diagnosis, no further evidence is really needed! In reality this rarely happens. It is recommended to get an MRI for further documentation and as a baseline. The dilemma occurs when the MRI is normal or very atypical. It takes a very confident neurologist to make the diagnosis at this point. this is the topic of a huge amount of discussion on the forum.
2nd Scenario
2 attacks
1 clinical lesion
The patient has had 2 or more clear-cut clinical attacks, by history, separated by remission. The doctor has found only one clinical lesion, that is one neurologic abnormality that can only be due to damage in the CNS. This same lesion may be found during both attacks. What the doctor knows is that there is "dissemination in time," because there have been two attacks. What is lacking to make the diagnosis is evidence of dissemination in Space, because only one part of the CNS can be shown to be affected. An example of this is two attacks and the only abnormality each time isoptic neuritis in the same eye. Dissemination in space can be determined one of in three ways
1) a Positive MRI (see definition above)
OR
2) 2 or more MRI lesions that appear consistent with MS in addition to Positive CSF findings (see above)
OR
3) The doctor can "wait and see" until the patient has a new attack with evidence that a new part of the brain or cord is damaged.
3rd Scenario
1 attack
2 clinical lesions
This patient has had only 1 clear attack and shows 2 abnormalities on neurologic exam that are consistent with MS. There is evidence that the disease has attacked more than one distinct part of the central nervous system, so we're okay on Dissemination in Space. There is no evidence that the disease has disseminated in time. This situation qualifies for the term Clinically Isolated Syndrome with a Multifocal Presentation. This person would qualify for early DMDs at this point, but most neurologists would want to see 2 or more MRI lesions as well before they made the decision to start early meds. To establish the diagnosis of Definite MS, the doctor would have to wait for one of two things to happen:
1) Positive MRI for Time requirement (see above)
OR
2) "Wait and see" for a second clinical attack. Remember that an attack must include objective evidence of damage.
4th Scenario
1 attack
1 clinical lesion
The patient has had only one clinical attack. The doctor finds clinical evidence of 1 lesion on the neurologic exam. This is also called a Clinically Isolated Syndrome. In order to make a diagnosis of Definite MS, the doctor must find evidence that there is both dissemination of space of the disease AND must also find evidence that there has been dissemination in time. The MRI and the LP become very important in this case, because there is no pattern of Relapsing andRemitting.
Note: At this point the patient has a Clinically Isolated Syndrome with a monosymptomatic presentation. The decision to treat early with DMDs may be made here.
Dissemination in Space can be shown by;
Positive MRI (see definition above for (Space)
OR
2 or more MRI-detected lesions consistent with MS in addition to Positive CSF results (see above)
Dissemination in Time can be shown by:
Positive MRI ( acording to the chart above for Time)
OR
The doctor can "wait and see" for the patient to have a second clinical attack
Note: At this point with both time and space requirements fulfilled the patient can be diagnosed with Definite MS. Note that quite a bit of time may need to pass (sometimes many years) before one of the requirements above is taken care of.
5th Scenario
Insidious neurological progression of symptoms and signs suggestive of MS
These patients present the hardest case for the doctor. They do not have the clear-cut attacks and remissions of the RRMS patients, so their history looks much like other chronic neurologic diseases. The doctor must rely on long-term deterioration and accrual of disability. In this case spinal lesions and a positive CSF become more important. In fact, the two of them together can substitute for brain lesions. In PPMS the majority of the disease is often found in the spinal cord. But the spinal fluid does not have to be positive as it was required to be by the first McDonald Criteria. This is in acknowledgement that there seems to be less inflammatory disease in PPMS, thus less of a tendency to form inflammatory immune antibodies (O-Bands) The diagnostic requirements for PPMS are:
** One year of disease progression. This can be done looking back at the patient's history, or by following the patient for a year and observing progression or a combination of both.
AND
** 2 out of the 3 following requirements:
1)Positive Brain MRI - this would consist of 9 T2 lesions or (4 or more T2 lesions + Positive VEP)
2)Positive Spinal cord MRI with 2 or more focal T2 lesions
3)Positive CSF (either +O-Bands or +IgG Index)
SUMMARY
Diagnosis is often an art, and doctors vary in their skill, their interpretation, their curiosity and their creativity. It is clear that MS can be daunting to diagnose. But, the process needed to begin the diagnosis is very clear. The neurologist must take a thorough history and physical, doing what is possible to make a timeline of the appearance of symptoms and their course. He/She must insert the observed "clinical lesions" into this timeline and make a judgment of whether the requirement that a disease has shown both dissemination in space and in time. From there he is ready to assess the MRI and decide which supporting tests need to be done and which tests will help add evidence in cases that are atypical and do not fit nicely into the patterns above.
Quix
Great information, Quix!
Thanks for clearing up the "9 lesion" myth. It's astounding that some neuros out there sound like they are going by this, and ignoring the McDonald criteria.
Every time I heard someone mention the need for 9 lesions, I was totally confused, since I was diagnosed as per the first scenario (2 lesions, 2 attacks). And as p*ssed off as I am at having MS, I do consider myself lucky in terms of how 'clear cut' my case turned out to be.
This looks like it took you a lot of time. It is really going to help a lot of the members here. Thanks for your efforts.
db
Thank you. I only hope it DOES explain some things. I realize how long and technical it still is, but when we get it up on the Health Page with all the formatting, I'm hoping that it will be clearer.
I want everyone to see that there IS, indeed, in one circumstance a possible need for 9 lesions. But, it sometimes sounds like neurologists are only applying that rule and disregarding all the evidence that there is to be had from the history and physical. In my case, I only had one brain lesion, but, I had lots of "clinical lesions" on exam. And I also had lots of spinal lesions - once they did the 3T MRI.
I hope everyone will tell me where the confusing parts are (after they read it three times, lol. I want it to be as good as possible when we post it for the HP. Also, it is in three parts here only because of the posting limit of 8000 characters. I think it will all fit on one HP. And there is a table (which I can't make on the forum) that will be clearer for some people.
I've been dreading doing this.
I want to start a section on Living with MS. I'm tired of doing the Diagnosis stuff for now. There are a whole bunch of things I've found on MS symptoms. Then I want to do some on symptom treatment, and maybe we can get some people to write up some of the alternative therapy stuff.
But, thanks for commenting!
Quix
Quix, I"m starting to understand,,,, yea really :) I sure appreciate you taking the time to do this as I'm sure all the rest of us are.
Quix I have to say the hardest part. well one of them is the "Wait and See"
I need to ask you If the subcortical and the juxtacortical is the same thing?
I know punctuate is pinpoint,, mine are not pinpoint.
Now I promise I read this 3 times and I'm sorry if this is a stupid question, but what can i say? I've got these darn Subcortical etc lesions in my brain watta ya expect??? lolol
(joke)) ha ha,,,
well gotta laugh sometimes hu?
Thanx
Ray
Ok, so know I have a question.
Is "punctate" not considered a lesion then? I'm assuming that punctate is < 3 mm. If I have 2 "punctate" lesions (corona radiata) and one large 1.5 cm x .0 cm lesion (periventricular), does that mean for a suspicion of MS, I only have one lesion?
Also, aquestion about the terminology of an "attack". If a person goes to a neuro with a bunch of Sx (major and/or minor), is that then considered an attack, even though all of those Sx are subjective and not clinical? To be called an "attack", does there have to be clinical evidence?
My neuro counted my first subjective symptom as an attack and included it in my diagnostic criteria. It was solely Lhermitte's Sign (which is not really a sign at all but a symptom, because it is subjective and unobservable by others). All I did was describe it to him, 1 year after the fact, and he called it my first attack.
db
oy!! I knew we'd get into the nitty gritty! Believe guys, I made this a simple as I could and it still took three posts! Every source I read was more vague than mine.
Ray - Subcortical is completely inside the boundary between the cortex and the white matter. BUT, I have read several places that radiologists have a great deal of difficulty distinguishing whether the lesion actually passes through the border or not, especially on lower power MRI machines. So, some lesions that are really juxtacortical are called subcortical. I don't know that this is true or if it is common.
Pat - First, the McDonald Criteria are pretty stringent as to what they allow for evidence. For the purposes of the Criteria a lesion must be >3mm to be counted. Does that mean a doctor can't take note of the lesions that are smaller and incorporate them into his own overall view of what's going on? Not at all. But, if a doctor lacks confidence or exerience with diagnosing MS, they can stay within the strict guidelines of the Criteria.
In absolute terms ANY hyperintensity, including a punctate one, is a "lesion" as it is an abnormality that can be seen, but it may not count toward your diagnosis depending on according to your neurologist. I don't think they swear any oaths to abide by the Criteria, lol.
For the Criteria they had to define an attack. So they did, and they were more stringent in defining it than people discussing MS usually are. They made it two part. The first part is what the patient tells the doctor - subjective part. But, in order for this to be beyond question, the Criteria also requires that an attack also have an objective part - an abnormality observed by the doctor. So, yes, "for the purposes of the Criteria only", an attack has to have a " clinical lesion."
In regular discussion about MS, this is not a requirement. We define our attacks by what is happening to us. Do you see the difference?
After the diagnosis, if you go to a neuro and have a whole bunch, or one, new symptoms, that would be considered an attack (unless your neuro is redefining things and making his own rules, like Rena's)
Do I make anything clearer or is it now even more garbled?
Quix :o
In terms of any individual neurologist, I think it is totally within their discretion to count a subjective report as a complete attack. That is where knowledge, experience, and confidence come in. If he were forced to defend the diagnosis he would not be able to do so with the Criteria, but so what. The major ramifications would be 1) your case might be excluded from a retrospective study where one of the requirements was strictly fulfilling the Criteria, 2) if you found out later you don't have MS (unlikely) he would have less of a defense.
Again, it comes down to knowledge, intelligence, experience and confidence of the neurologist.
Quix
Kudos! It's great to see this all laid out, along with scenarios that illustrate the points. It's a lot, but I don't think you can make it simpler.
sho
What you say makes sense. I think that may be why my neuro wanted me to hold off on starting DMD's until I was accepted in a program that provides full coverage of the cost of my meds (annoying since I didn't want to wait and already have 90% coverage at work, but that's another story).
Eligibility for the program is decided by a committee of neuros, and I was told the only reason someone might be denied coverage is if the committee disagrees with the diagnosis. So maybe he was thinking they may not agree with his findings. As it turns out, I was accepted for full coverage, so apparently the committee did agree.
db
OK, so I have a totally different scenario...Mine! :)
I have lesions up the......um...wazoo, and only 1 documented 'attack', with several "subjective" symptoms...(some still left over from end of March attack)
BUT....the majority of my Neurological exam was normal.....The only 2 things I had back in December were slow corneal reflex in right eye, and I bombed the heel-toe test...(Sorry, I don't know the technical term for that one)
What does this mean for me? I go to the MS Clinic in 10 days!!!
Tammy
Hi, Tam-Tam,
I can answer questions in general about how these criteria can be applied, but I don't think it is a good idea to try to apply them to people's situations. I don't have all the facts and well...., I'm just uncomfortable doing it.
Is that okay?
Quix
this is what my mri results said....
There are two nonspecific foci of increased signal seen on FLAIR in the left cerebral hemispheres both of which are in the left frontal lobe and subcortical WHITE MATTER measuring approximately 3.3 to 3.8 mm.
Impression:
NO evidence for acute ischemia
There are two nonspecific foci of signal withing the WHITE MATTER in the left frontal lobe in the subcortical region. this is nonspecific but atypical for demyelinating lesions these can be seen with migraine headaches and chronic vessel ischemic change.
OK, so I'm understanding... lol
this is what my mri said. (above) sooooo my understanding is that mine are actually in the juxtacortical area because he stated they were in the white matter,,, and not between...
Do I have it?????? Have I struck gold??????? am I a genius??????? lolol
(Joke again)
one more question,, probably stupid but here goes..... Is the cerebral hemispheres and the cerebellum same thing??? oy!!!!!!!!!!!!!!!!!
I hope you start feeling better Quix I'm sure your exhausted you've worked so hard on this and Quix it is amazing to me. You are helping sooooooooooo many people and even though you are not actually working in the clinic, Quix your working your guts out on here, It is sooooooo appreciated and needed. Your a very Wonderful/Selfless/kind person.
Prayers to you
Ray
Ohhhhhh....I wasn't really looking for any answers when I posted that....It was just more or less just to add in a totally different scenario...
I think it's fascinating to see (read) what different stages a lot of us Limbo landers are in and I just wanted to add my 2 cents. :)
My question was more of the hypothetical type.....
It's also extremely interesting to me the difference of opinions in Neuro's as far as following the McDonald criteria....It seems no 2 Neuro's are the same....
Have a great day! :)
Tammy
No, I'm afraid you have not struck gold. :( The description juxtacortical refers to crossing a thin boundary not lying between the white and gray matter. There is not "in between".Sorry. You're stuck with sub-cortical, in the white matter.
To say that they are in the juxtacortical position, the radiologist actually has to say it.
Thanks for the kudos. Yes, this was very hard to distill, and I see that I have more work to do on it. There is just so much info and so many specific definitions. Some of the definitions here are used only here and are different from common usage. Alas...
Tammy, sorry I didn't see your point. Yes, neurologists will have a different take on the Criteria often if they are putting more weight on one area than another.
We know there are strict "lesion counters."
We know there are nurrows that think EVERYONE has to have 9 lesions
We know there are docs that look at someone with several clear attacks and accumulation of disability and will accept less than the full MRI criteria (mine for instance).
We know that there are nurrows that think a positive LP is mandatory. I really wonder where they see that in the Criteria. (Actually I know they get it from mis-interpreting studies done AFTER the Criteria were published).
To all: Please note here or PM me where my descriptions are not understandable. I want this in GOOD form before it graduates to HP status.
Thanks all,
Quix
Thank you for laying this out in laymen terms. As much as you possibly can. With all the if, ands, and buts, its a wonder anyone gets dx.
I just wanted to let you know you are so appreciated here. I know it is exhausting and very time consuming to put all this together. It shows what a selfless person you truly are. Taking time for others to understand this diease.
BRAVO TO YOU!!!
nikki - Thank you so much!
I bumping this up, because it is such important information and I don't want interested people t miss it!
Quix
I wanted to thank you for doing all of this work on the criteria. I really appreciate how you broke it down. I have been doing alot of reading in the last few weeks since my Neuro gave me a "probable MS" diagnosis.
I still have a question..... I didn't present with clinical symptoms, but instead had numerous lesions on my first MRI, and then showed more lesions on my 2nd MRI at the 6 month mark. It was all by accident that this was discovered. My only symptoms so far have been dizziness of and on for the last year and the Nuero noticed a slight left hand tremor on exam. I can't really establish a patterns on the symptoms coming and going yet. My LP, VEP, EEG, and bloodwork have all been normal. My Neuro is reluctant to officially diagnose MS because of the lack of clinical symptoms. If I read the criteria right, you really need the clinical presentation for a diagnosis. Is this correct? What about those of us that are "atypical" and don't neccessarily have symptoms that have shown as "attacks"?
I feel like with all of the reading I have been doing lately to get up to speed, that I should have just gone to medical school years ago. )
My Neuro's office called today with my schedule. They have me doing another MRI in 4 weeks, with an appointment with him a few days later. That will make my 3rd MRI in 8 months. Yippie!
-Amy
Would you post this question on the front page. We have another person posting who had her lesions discovered by accident. It's a great thing to discuss.
Quix
As always, Quix, a great thread. Here's another scenerio for you. What about using other test results to count as an objective sign? For instance, I had an ENG which showed an abnormality in the CNS, not from the ear itself.
I also had neuropsych testing showing problems similar to those found in MS. And, I had urodynamics testing that showed a slow flow rate and retention. The urologist has stopped short of calling it a neurogenic bladder, but he goes on to say he is treating me as if I have a neurogenic bladder. I'm suppose to be taking Flomax (need to get back on that again).
Thanks for all of your help.
Thanks, Quix. I can see where I have documented clinical lesions, but I think the neuro has lost sight of that. He seems to only focus on what he sees in the exam at hand without remembering what showed up (and then resolved) before..
I really recommend for all people in Limbo - Read this Carefully. It explains a lot!
Quix
Thank you, Thank you, Thank you, so much for putting this together.
Finally something that makes sense with out the medical stuff that you can't even start to understand. I feel like I am in a better place now that I know a little about the system of being diagnosed.
I am going to use this post as reference!
Thanks again,
Aura