Is the MS lexicon in need of an update?

I

Barts-MS rose-tinted-odometer: ★

In the last 2 days, you will have seen from my posts and the discussion that it has generated that there is a problem with the current MS lexicon. We need to get some form of nomenclature that addresses the confusion about what MS is as a disease and at what is the state and/or stage of MS for individuals with the disease. Knowing how to refer to people with MS is important for several reasons. It affects how they are managed, it has implications for what treatment pwMS are eligible for and it has wider implications for the MS community. The latter refers to clinical trials and for getting MS DMTs licensed for treating MS.

What best describes your MS?

Active radiologically isolated syndrome
Inactive radiologically isolated syndrome

Active clinically isolated syndrome
Inactive clinically isolated syndrome

Active relapsing-remitting MS
Inactive relapsing-remitting MS

Relapsing multiple sclerosis
Active relapsing multiple sclerosis
Inactive relapsing multiple sclerosis
Active relapsing stable multiple sclerosis
Active relapsing worsening multiple sclerosis
Inactive stable relapsing multiple sclerosis
Inactive stable worsening relapsing multiple sclerosis

Active secondary-progressive MS
Inactive secondary progressive MS
Active stable secondary-progressive MS
Active worsening secondary-progressive MS
Inactive stable secondary progressive MS
Inactive worsening secondary progressive MS

Active primary-progressive MS
Inactive primary progressive MS
Active stable primary-progressive MS
Active worsening primary-progressive MS
Inactive stable primary progressive MS
Inactive worsening primary progressive MS

Early-stage MS
Late-stage MS
Advanced MS
Smouldering MS
MS with hidden disabilities
MS with hidden progression
MS with silent progression
MS with progression independent of relapses

Benign MS
Malignant MS
Indolent MS
Highly-active MS
Rapidly-evolving severe MS

Etc…

I am interested to know if all this makes sense to you? I am sure I have also left some descriptors off the list. If I have please let me know. 

CoI: multiple

Twitter: @gavinGiovannoni  Medium: @gavin_24211

About the author

Prof G

Professor of Neurology, Barts & The London. MS & Preventive Neurology thinker, blogger, runner, vegetable gardener, husband, father, cook and wine & food lover.

27 comments

  • I’m currently described as having indolent MS! How insulting when I am constantly busy . Prior to this I’ve been told I have benign MS and that I’m asymptomatic. Prof G, you’re so right. Let’s abandon this labrynthine nomenclature for one overarching term, then we will need descriptors. But salami slicing MS down like this only benefits Pharma, not patients, medics and research. The NHS Is paying more than it should for DMDs and their development is hampered. Rollout is slowed and we seem hostages to fortune and the US Orphan Drug Act.

  • I’m being treated with 6 weekly infusions of Tysabri, the infusion does nothing for my symptoms but it stops progression. My MS is relapsing – remitting so I really don’t know where I would be on the revised version of MS

  • I think there needs to be a consensus of MS terminology and exactly what the stages of MS are but it must come from an agreement amongst neurologists. Until there is international agreement MS will defined differently within borders. Good science and medicine should be universal. We don’t want MS to be like the analogy where blindfolded men are touching different parts of an elephant and each exclaiming what it is.

    • Yes Steve, this is where we are, and where we have been for the last (approx) 180 years.
      ‘There is a true missing link in our understanding of MS and we must be open to surprises’ (Bruce Trapp. Ann Neurol 2004.

      We will continue to be chasing our tails until the missing link is found and the mechanism of MS is discovered.

      Perhaps we need an international review of the likely ‘missing links’, followed by a really serious programme to pin the bas…d down!

      • Do we really think that if you put the great and the good in one room you would get a missing link…herding cats is easier. It is also easier to get a response to someone elses opionion than make one for consideration, I suspect

  • Perhaps the descriptors should come from the patients who live with the disease every day. I would suggest: quite sh*t MS; sh*t MS; really sh*t MS; really f***ing shit MS This would capture the impact the disease has on a patients lives – the pain, the losses etc. I admit the descriptors aren’t based on any biological processes, but they capture the impact of the disease more than, for example, inactive Primary Progressive MS, which sounds quite harmless.

    As Shakespeare said “What’s in a name?“ Rather than waste too much time on words, let’s get trials done on the underlying disease. We should really be in a position where the only terminology we use is “the disease formerly known as MS which, like smallpox, has been eradicated through global vaccination efforts”.

    • Sid, I’ve disagreed with several things that you’ve said on here in the past, but your last two posts have been spot on. Forget all the other terminology, these are perfect (plus I would happily donate to Prof G again if we had video evidence of him introducing them as the new universally accepted descriptors in a webinar without smirking).

      The term “inactive” just seems to mean that doctors have a reason not to start a patient on or change their drugs. And anyway, if we all have smouldering MS mashing our brains quietly in the background and the MRIs are not picking up tiny or grey-matter lesions, aren’t words like “inactive” or “benign” misnomers anyway? Also unhelpful are things like “you only have…” or “it’s just…”. Feeling guilty that we may be making a fuss or time-wasting doesn’t make anyone feel good.

      I agree with Fabio, for me it’s enough to know that I have MS, and as many others have already said, my only wish is that scientists and academics find effective treatments for all PwMS (however far along we may be) which target the underlying disease and repair the damage that has already been done. If MS healthcare professionals can’t even agree on the terminology and what the definitions of these terms are, what chance do we have of getting anywhere with that?

      • Thank you!
        Also the term inactive can interpreted as “you don’t have activity therefore you do not need DMTs”. And to compensate you will get relapses back and other neuronal pathways damaged.
        This is what scares me the most in particular now that more high effigy drugs are available. I believe this will happen to just a minority of patients but even just one patient will be too much.

  • I don’t think that the name for the thing changes the thing itself.

    Most people with MS have a huge notebook or file that describes their descent into the maelstrom.
    One trite phase, no matter how technically correct, or descriptive will help their treatment.

    While the medical world has become entangled with the commercial and political worlds in trying to provide care at a cost constraint, the focus should be on the real problems.

    One real problem is that the MS patient is likely getting worse over time. Their chronic disease is slowly eating up their nerves and disabling their muscles and wreaking havoc everywhere in their body. They are getting old much faster than their friends and peers. Their medications are too expensive and they don’t work well enough. They don’t have enough access to treatments that will alleviate their symptoms.

    If their doctor is treating them by deciding which bin they fall into, and what DMT they are or should be on, HEAVEN HELP THEM ! That is just scratching the surface.

    If politics or regulatory issues are blocking giving good care, then this should be addressed head on.
    I know that this is your mission and you are making good progress.

  • OMG !

    Yes, we need to de-clutter. And fast.

    One disease, and a million ways to slice it. It doesn’t cut it.

    I agree. And, RIS does NOT exist. Back then, when it was first described in 2009, CSF studies were not considered (so a patient has lesions in the brain that correspond to MS, the exam is unrevealing and I guess it was dubbed RIS). However in this day and age, we have to do CSF studies to worry about DIT criteria or C-spine for DIS criteria and consider McDonald criteria to correctly diagnose MS, that which was previously diagnosed as RIS.

  • I like this beginning.
    My diagnosis would be Inactive Relapsing Remitting Multiple Sclerosis
    “Inactive “ has a positive spin. My MS Team, Pharmaceuticals and I are responsible for that.
    It’s motivating for Me.
    My Insurance can justify covering expensive drugs and treatments.
    The Early,Late,Advanced,Smouldering labels and Hidden Silent labels
    Could be put into numbers, code, or sort differently.
    Examples: age of onset/duration/#exacerbations/#deficits-losses-Dysesthesias /On DMT/Hidden Y/N
    49/13/3/3/Y/Y

    Just a few thoughts.
    M

  • I couldn’t actually care less what you want to call my “progressive MS”. Or more sticking plaster DMTs or mouse studies. I am interested in getting to the true root of the disease, antivirals.

  • My neuro simply says “MS with progression.” I have never had a relapse but I don’t fit the typical picture of PPMS as a spinal disease. Instead I have first symptom of cognitive decline, followed by fatigue, followed by gait problems and weakness, followed by deteriorating right hand function, now severe tremor.

  • “Nerves” are being hit here. But a good question. I fall into a category of no MRI evidence for slow clinically progressive disease, after a relapsing course. 25 years on interferon beta 1b, then a quick stint on Ocrevus until covid. Biggest complaint concerning names is, I had to dig to find out that I am far from alone, but there is little recognition for it, seemingly due to a clear lack of recognition that this is what (unless I am misreading something) commonly occurs. Wish I had been told to expect it rather than allowing myself to believe that no MRI evidence meant everything was OK. So to specifically answer the question, words like active, inactive, silent, have to be better defined. Am I active, inactive, silent? Still don’t know what to call it but I know I am not alone and that it is typical.

  • Just multiple sclerosis?

    If we sum the content of the posts since I began reading the blog (Feb 2020) most of the definitions goes one against the other.

    We do not need to classify the disease but just to describe the patients for example to do subset population analyses (eg poor representativeness of EDSS for >6.5 so longer trial needed for them)

    To me, if a patient has MRI activity as new lesions or expanding lesions he needs a DMT. If on a DMT and he has no activity than the DMT is keeping MRI activity at bay and should not be discontinued, if on a DMT and he has activity it is time to escalate to higher efficacy, if possible.
    If a person has no MRI activity at all (and never had since diagnosis, do these cases exist?) then I would treat as those people with activity as they are a minor number of pwMS and possibly we are trying to improve their outcomes. Also, the fact that MRI is not detecting activity it does not mean that there is no activity, maybe the technique is not sensitive enough to detect it (like reported in the post on post-mortem detected cortical lesions that are missed by MRI) and they fall back on the point above.

    Finally, for all of them drugs targeting progression are needed for neuroprotection, switching off hot microglia, clear oligoclonal bands, brain preference etc.

    So, one name and full access to all DMTs.

  • Was diagnosed with active relapse remitting, I think im currently benign though…I have no symptoms but thats because of Tysabri

  • Technically, I have inactive RRMS – MRIs stable but it is completely misleading as to where I am currently at.

    I have been gradually declining since I had to switch from Gilenya to Ocrevus 2.5 years ago. I start Lemtrada in 4 weeks. I feel like crap but am still a highly functioning professional. I just want to be on a DMT that doesn’t give me so many side effects that I become despondent and cannot face maintaining therapy and every other usual part of staying active, engaged and independent.

    I would personally like to see smouldering MS recognised and studied. It is real and this is the only site I have been able to read something that resembles my own reality.

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