Friday, 26 August 2011

Fulminant MS

Epub ahead of printRohani & Ghourchian. Fulminant multiple sclerosis (MS). Neurol Sci. 2011 Aug 24.

Fulminant MS is the most malignant form of MS which usually leads to death with in a few weeks.

Fulminant MS  can be accompanied by optic neuritis (ON); however a long interval between ON and the fulminant phase has not been reported. 

This is a case report of 30-year-old woman with a history of ON that occurred 1 year ago; who tragically developed rapid deterioration in her MS. She went into to a unresponsive or vegetative state followed by a seizure. All investigations were compatible with a diagnosis of MS. 

"We don't usually present case reports on this blog, however, this case illustrates that MS can be very severe."

21 comments:

  1. I'd like to know more about Fulminant MS. What is the pathology of this particular brand of MS and why does it affect young people? Is Fulminant MS a less protracted version of PPMS?

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  2. Complete blockage of her veins?

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  3. Re "Complete blockage of her veins?"

    As I understand it, Marburg's Variant, or Fulminant MS if you prefer, is where the axons are severely attacked. PPMS is in ways like Marburg's Variant I suppose, or at least can be, though a way slower version.

    To be honest I don't know enough about it. I would like further info.

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  4. Prof G,

    Very sad case. I find it staggering that in 2011 the medical profession can't help a relatively young person like this. It's easy to get carried away with so-called research breakthroughs, but the real test of any medical specialism is whether a disease can be stopped in its tracks, reversed, or not be life-threatening. Unfortunately, neurology fails on all three. While there is certainly interest in MS research findings, cases like this (and Debbie Purdy etc etc), really show how little progress has been achieved. Apart from some pretty ineffective injectibles, and a more effective drug that can give you PML, the reality is that we are years away from treatments which will benefit MS patients, particularly those with progressive MS.

    My plan is to re-visit this site in 5 years time and hope that we have moved beyond the mouse stage of research.

    Note - my comments relate to all MS research not your specific team. It just frustrates me that at the Ectrims/Actirms conference in October there will be plenty of backslapping and awards dished out for excellent research, but this case (and others) is the real test of where we actually are!

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  5. Totally agree with the above comment. This post scared me. MS is such a horrible illness I feel angry that scientists have been so slow in generating effective treatments. By effective treatments I'm talking about drugs that help combat progression. MS is a death sentence; a slow lingering curtailment of function and quality of life. A part of me wishes there would be a sudden spike in the number of people who develop the disease just so that scientists and health policy makers will pull their finger out and do something useful. The problem with MS is that 2.5 million people in the world is not enough to attract greater investment.

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  6. Too long spent on the mouse model, too long on small projects to get Drs their PhDs, too long from an theory to a trial (EBV role was first considered 30 years ago), too many sufferers have died waiting for a breakthrough. Nothing for those with progressive MS. And still no timescale for treatments which really work. Cancer treatments just keep hitting the shelves. MS treatments are turned down as not cost_effective. Something is rotten in the state of MS research.

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  7. Re: "Complete blockage of her veins?"

    The "complete blockage of veins" is called cerebral venous sinus thrombosis, which may or may not be associated with deep and/or superficial cerebral vein thrombosis. This condition is completely different to fulminant MS and easy to differentiate on MRI. These are completely separate conditions.

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  8. Re: "PPMS is in ways like Marburg's Variant I suppose, or at least can be, though a way slower version."

    No Marburg's variant is distinct from PPMS. Marburg's variant is typically rapidly progressive and is associated with large inflammatory lesions on MRI that look like tumours.

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  9. This comment has been removed by the author.

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  10. Re: "Something is rotten in the state of MS research."

    Are you an old hack? You are clearly a cynic. If you have been following this blog from the start you will have noticed the highs and lows of its readers.

    On balance there is a lot of good things happening in the field, with several breakthroughs in the last year alone.

    Prior to the modern era of instantaneous news and the empowering of MS'ers with knowledge this debate would not be happening. Science is a slow, but incremental process; what is reported today may have a major impact on the field in the future. One example is the first betaferon/betaseron study in the early 1990's; who would of thought that treating early with interferon-beta would have an impact on MS survival 20 years later? Is that not an advance? Just imaging what the impact could be from the more effective emerging therapies.

    All we are trying to do as a group is keep you up-to-date with what is happening in the field in a form that is digestible (excuse the pun) for the majority.

    If you missed the interferon-beta survival post here is the link:

    http://multiple-sclerosis-research.blogspot.com/2011/05/ms-and-survival-long-term-betaferon.html

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  11. Re: "I find it staggering that in 2011 the medical profession can't help a relatively young person like this."

    This is a very unusual case and I agree it is very sad. However, most of the time we can do something to prevent such a poor outcome. I think we have come a long way; "therapeutic nihilism" has given way to "tempered enthusiasm" regarding MS treatment. Several of my neurology colleagues specialising in other disease areas often express their envy at how far and at how quickly we have advanced the treatment of MS. I don't understand why you are so cynical! My team and I are not; we are very positive and upbeat about the future of MS treatment. Whether or not it is progressive MS or MS prevention we will continue to burn the midnight oil.

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  12. Prof G, optimism and positivity are so important. Please keep up the good work. In G we trust!

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  13. "I don't understand why you are so cynical!"

    We are just coming at this from different angles. A patient's perspective will always be different. I've no doubt that your team and other teams are working very hard and that in the future there will be more advances. But we have a generation of suffer who are SPMS, PPMS, have lost their jobs, can't aprticiapate in sport any more. How optimistic or positive should they be I have a friend aged 23 with SPMS (very aggressive MS at Uni following shortly after a bad case of Glandular Fever!). Her parents have moved into a bungalow and wasted their savings on Aimspro and LDN. Her case won't be unique. You are no doubt right that future generations with ealry RMS will have a much better outlook. But there's still 50,000 people in this country with progressive MS and, apart from an annual neuro check and some pain relief, they are not offered any treatments to stop or reverse the progression. I test science (medical science) by results. At present I don't know of any treatment to stop progression, repair some of the damage or prevent MS from happening. I knopw that you and your team are positive about the future, but 5-10 years is a long time for someone with this disease. The point of my post:
    - to express my gratitude for the work you are doing.
    - to present a more cynical view (remember cures have been promisedd since the 70s), and hope tht I am proven very wrong.

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  14. The person above is right. People involved in research expect MS’ers to be grateful they’re bothering to invest their time in MS investigation as opposed to some other illnesses.

    The case point of your 23-year old friend almost mirrors my own experiences. I got PPMS in my 20s and it’s pretty much robbed me of all quality of life. Furthermore, my retired parents now have the additional worry about how bad I’ll get. They feel so helpless watching their young son just worsen and not being able to stop it.

    I’m not a cynic; I am a realist. There is nothing there on the market for progressive MS and we are years away from effective treatments. Time is not on our side Prof G. Hopeful this gives you some context to our frustration and vitriol towards a medical science that is, currently, failing us greatly.

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  15. Re 'People involved in research expect MS’ers to be grateful...' :
    We should be grateful, whether or not they expect it. Their work will lead to results some time in the future. It's sad that it may be too late for many of us. Unfortunately medical science is not magic.

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  16. The researchers are the ones who should be grateful....they are getting funded (salaries paid) through our taxes and charities...We are keeping them in a job.
    Our gratitude will come when prof and team g find the cure...

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  17. Re: "People involved in research expect MS’ers to be grateful they’re bothering to invest their time in MS investigation as opposed to some other illnesses." AND "The researchers are the ones who should be grateful....they are getting funded (salaries paid) through our taxes and charities...We are keeping them in a job."

    Several people who work in my group have a very personal connection to MS; their involvement in the field is a lot more than simply having job.

    On balance we all enjoy our jobs, for which we are grateful.

    On a more personal note: I always tell my daughters, "there is no greater privilege in life than having a job that you enjoy; a job that makes you want to get to work in the morning and work late at night, a job that consumes you, a job that makes you both happy and sad, and a job that you believe makes a difference". This is how I feel about my work and for that I am truly grateful.

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  18. Gloating about the joys of employment on a blog read by people whose MS has robbed them of their ability to work??? This is about typical, in my experience, of the understanding of neurologists.

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  19. I have had the pleasure of meeting many of Team G and what has struck me about all of them is just how passionate they are about helping MS'ers. It's not an easy field to be in and can be just as frustrating for them as it can be for MS'ers in terms of the relatively slow progress, which I understand can largely come from bureaucracy and having to tick all the right boxes. MS'ers SHOULD be grateful, as without these incredibly hard working researchers there would be no hope at all and that would put MS'ers into an even worse predicament.

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  20. Should we be grateful to everyone at tescos and doing their job by getting us food?

    Bureaucracy? Hmm...why is it that other research groups seem to be getting things done? Team G don't seem to be making the news or top tier scientific journals with their work....but they are making fruit videos

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  21. Re: interferon-beta

    Last year’s news, but still relevant today:

    http://www.independent.co.uk/life-style/health-and-families/health-news/ms-drug-trial-a-fiasco-ndash-and-nhs-paid-for-it-1991104.html

    “MS drug trial 'a fiasco' – and NHS paid for it.”

    "The most expensive publicly funded drug trial in history is condemned today as a "fiasco" which has wasted hundreds of millions of NHS cash and raised fresh concerns about the influence of the pharmaceutical industry…

    …The first report on the outcome was due after two years but was not published until last December (2009), seven years later. It showed that the drugs failed to delay the onset of disability in patients – defined as walking with a stick or using a wheelchair – and may even have hastened it…

    ….There were 5,583 patients in the scheme at a cost to the NHS of around £50m a year, amounting to £350m over seven years to 2009. The Multiple Sclerosis Society said twice as many patients were using the drugs outside the trial. That implies a total NHS cost of £700m for a treatment that does not work….”

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