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Primary progressive and steroids / Fampyra

I was diagnosed primary progressive eight years ago. I’m 59. I asked my doctor to prescribe Fampyra (the walking drug) which he did. In conversation, I told him that my mobility and fatigue had been worse for at least 6 months and felt like I’d had a cold all this time. He suggested I might still have residual inflammation and prescribed predisolone steroids 50mg daily for two weeks. I started both drugs last Friday. What an amazing improvement ! 1. Foot drop mostly gone 2. Bladder urgency almost gone 3. Night spasms almost gone 4. Biggest benefit - fatigue much reduced. I have always understood that steroids weren’t used to treat ppms and are ineffective in ppms. Have any other ppms people experienced steroid treatment with good results? I’d like to think that it’s the Fampyra that’s helping and not a temporary steroid effect. John H

It could be the Fampyra - when I started it I saw an improvement in lots of functions and a big reduction in tiredness. It wasn’t as dramatic as your improvement sounds however. The other possibility is that your diagnosis was wrong and the steroids have helped, which would mean you have RRMS. What did they look at to diagnose you with PPMS?

My diagnosis of ppm s seems to have evolved rather than been stated. Initially, I had the usual MRI, evoked potentials and lumber puncture. Neuro (who I haven’t seen for five years) said that there was no point in seeing him again as there was no treatment for ppms. My ppms has just been assumed. I’ve always found that any infection, tummy upset, poor sleep or a cold really affects my mobility, but I usually recover quickly.

I think the normal way to diagnose PPMS would be to have a gadolinium enhanced MRI done when you are in the middle of a deterioration. If this shows areas of inflamation, then you have RRMS. If there are no areas of inflamation, you have PPMS. Another way that a good neuro would be able to tell is you will be given steroids and if you improve then it is likely you have RRMS. I don’t know what you do if you get a diagnosis that seems to be wrong. My experience with a rubbish neurologist was that he was extremely touchy and defensive - he didn’t take at all kindlly to suggestions that he might have made a mistake.

I would say that starting you on steroids and Fampyra at the same time shows your neurologist is poor because you now don’'t know which one caused the improvement you have seen.

You could try stopping taking the Fampyra for a few days and see if the improvement goes away (I had a lot of trouble getting up the courage to do this). If you don’t deteriorate then it is likely the steroids have helped you and you have RRMS. I don’t know what you would do then. Maybe other people on the forum might have suggestions?

Diagnosing MS relies on the McDonald criteria, namely:

PPMS: at least a year of worsening symptoms (not more symptoms, although there may be, but symptoms getting progressively worse) and two of the following: at least one brain lesion in an area typical of MS, at least two spinal lesions, a positive LP.

RRMS: at least two attacks and at least one lesion in at least two of four places typical of MS.

Assuming the neuro follows this, I cannot see any way of being misdiagnosed with PPMS when you actually have RRMS.

Although the level of inflammation is less in progressive forms of MS than in RRMS, it is still there. However, I’m not convinced that a (relatively) low dose of 50mg a day would do much to counter this - I’m not a medic and could easily be wrong, but perhaps the steroids are suppressing an infection or something else that might have been exacerbating your symptoms? (That “cold” you felt you had all that time?)

People with RRMS can have MRI scans with gadolinium during a relapse and have no visible lesions. There are many reasons for this, the main ones being that MRI is not perfect and timing.

Similarly, steroids do not always work in RRMS. There are many reasons for this too, the main ones being individual responses and timing.

Karen x

There is absolutely no reason to think that the steroids working means a misdiagnosis at all. Steroids are used in PPMS and in SPMS to great effect. Although it is not done much (if at all in the UK) steroid treatment is very common in progresive MS in the States. A monthly dose of IV steroids is given on an ongoing basis to help deal with symptoms and give relief. It is called pulse steroid treatment and has also been shown in some studies to suggest it may delay disability.

Research to date has focused on whether long-term pulse IVMP therapy may delay the progression of brain atrophy or disability in patients with progressive disease. In summary:

u A 5-year, phase II clinical trial of IVMP in patients with RRMS (Zivadinov et al., 2001) demon- strated that prolonged treatment with pulsed IVMP slowed the rate of whole-brain atrophy, the development of destructive lesions (T1 black holes), and the development of sustained physical disability as compared to controls.

u Pulse IVMP prevented the development of brain atrophy in 11 patients with primary progressive MS (Pato-Pato et al., 2003).

u A small retrospective study of patients who had received monthly pulses of IVMP showed that the treatment was associated with improvement in fatigue, spasticity, and motor strength (Pirko and Rodriguez, 2004). No acute exacerbations occurred in 9 of 10 patients with PPMS or SPMS.

u Pulse IVMP has also been shown to prevent sustained disability. In a phase II study of 108 patients with SPMS, IVMP therapy was associated with a marginally significant delay in the onset of sustained disability (Goodkin et al., 1998).

u In contrast, a single course of IVMP during an attack of acute optic neuritis failed to prevent the development of optic nerve atrophy (Hickman et al., 2003).

https://www.google.com.au/url?sa=t&rct=j&q=&esrc=s&source=web&cd=4&ved=0CEEQFjAD&url=http%3A%2F%2Fwww.nationalmssociety.org%2Fdownload.aspx%3Fid%3D553&ei=Qxu7UfSsNunsiAfhu4GoDQ&usg=AFQjCNEZgctoz6WcGY4JSIZoajNNK3PXNA&sig2=YzYrYthcqvltV9EEIvka9g&bvm=bv.47883778,d.aGc&cad=rja

Cheers,

B

I see you`ve changed your username back to brog54, from Possum saver, yet your avatar is of the little fella. Can I ask why please?

luv Pollx

Bum,

Sorry, bump!

Hi, I dont have a dx yet. Had my 3rd MRI in 3 years yesterday and my LP has been clear. I had steriods 3 years ago and they removed all sensory symptoms completely (never had them since) but didnt improve spasticity which gives me drop foot. I hope that it is the fampridine that as helped you as I am hoping to try it soon.

Take Care

Moyna x

I’m afraid I can’t understand the McDonald criteria. Can you explain them to me Karen? It’s how they’re used to diagnose PPMS that i find confusing. I’ll use myself as an example. I had something like an attack in 1999. My balance was quite bad and I reacted very badly to heat. I went to my GP and she found I had some odd reflex reactions. I had an MRI and was told I almost certainly had MS because of how it looked. For the next seven years I saw a slow but pretty steady deterioration in my walking. Presumably any time during these years, if I had asked I would have been told I had PPMS, if I had asked? In 2007, I had my first proper attack, when I could walk, but only 100 yards or so. After a few weeks, I got better but wasn’t able to walk as far as before the attack. This has happened with increasing frequency over the years, more attacks, losing more function and getting some of it back but not all. Like I say, don’t the McDonald criteria give me a diagnosis of PPMS? But later, it becomes obvious that I don’t have that? And if this is true for me, could it not also be true for johnh? And other people? Or am I missing something? Please explain?

@Sewingchick: My understanding is that, assuming that the diagnosing neuro knows anything about MS, it is not possible to go from PPMS to RRMS. If it were to happen, I would think that someone with a history of progression and a PPMS diagnosis who begins to have relapses would have their diagnosis changed to PRMS (or to something other than MS). Whether or not you could have been diagnosed with PPMS in 1999/2000 would rely entirely on the degree of progression and your MRI and LP results at that time. Without that information it remains speculation. However, it would clearly have been the wrong diagnosis as your history from 2007 is classic RRMS. This does rather beg the question whether or not your “something like an attack” in 1999 and subsequent walking problems were related to your MS at all. I guess we’ll never know.

If you would like to better understand the McDonald criteria then I recommend reading the original paper. It is fairly easy to follow and is available to download, free and in full: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3084507/

Kx

My point would be that, in some cases (like mine), a person’s MS can start off looking like PPMS but later on it becomes clear that it is actually RRMS. When I had my second proper attack, I asked a neuro why I had started to have attacks when, for the seven years since my diagnosis, I had just seen a slow deterioration. He said “maybe your disease is moving into a new phase”. To him this was neither surprising nor unlikely.

I think I was lucky that I was never given a label when my MS was in its earlier phase because, if I had been, I wouldn’t be eligible for DMDs. I’m on Tysabri now and I would say that my rate of deterioration has slowed quite a lot. It seems to me that johnh may be in the same position as me but if he is, he is unlikely to ever get his PPMS label removed and so he will never be eligible for DMDs.

I repeat, whether or not you could have been diagnosed with PPMS in 1999/2000 would rely entirely on the degree of progression and your MRI and LP results at that time. Without that information it remains speculation. However, it would clearly have been the wrong diagnosis as your history from 2007 is classic RRMS. This does rather beg the question whether or not your “something like an attack” in 1999 and subsequent walking problems were related to your MS at all. I guess we’ll never know. I will just add, though, that although progression is usually overshadowed by relapses/remission in early RRMS, it is still active in most people - and the degree to which it is active varies by person.

Since you raise the issue of DMDs, one of the key eligibility criteria for DMDs in the UK is having two clinically significant relapses (ie acute attacks that cause significant impairment / disability / debilitation) in two years.

Ultimately, therefore, if someone is having clinically significant relapses, then a diagnosis of PPMS is inappropriate. If someone is not having clinically significant relapses, then questioning their PPMS diagnosis is almost certainly inappropriate.

@John H: my sincere apologies for my role in your thread going off topic. I hope your improvement has continued and that Fampyra continues to work wonders :slight_smile: Kx

Update after steroids and two weeks on Fampyra. Having had remarkable improvements during steroid treatment, I finished the two week course last Thursday, Friday saw almost all symptoms disappear. Miracle! So good I booked a restaurant for dinner on Saturday night. First evening outing for two years! However, deteriorated during Saturday to the extent that I could only take a few steps without resting. My wife had to help me into bed. No night out! Improved over Sunday to 80o/o of steroid time condition. Still better than two years ago, though. I’m hoping that the Fampyra, which must have helped a bit, will continue to allow more improvements. I understand that maximum improvements only appear after 6-8 weeks. My nerve pathways must still be able to function. Last Friday I felt ‘cured’! Best wishes John H.

There is also PRMS that is a possibility http://www.mult-sclerosis.org/progressiverelapsingmultiplesclerosis.html

Hi John,

PPMS is much harder to dx than RRMS and I have read that people have been misdx with PPMS frequently when it has turned out to be something else entirely. My neuro is very reluctant to dx someone with PPMS if they have a clear lumbar puncture. This is also mentioned by the Prof on the Barts MS Blog as he believes MS with a negative LP is an entirely different disease. I dont have a dx of MS as my LP was clear as is my brain scan. I do have a lesion on my cervical cord. I think I fit the PPMS picture better as my walking is the only issue - although the whole progression was started by some sort of attack/relapse nearly 5 years ago.

I was told the postive effects of steriods last 6 months so stopping them for one day should not have effected you. Although I must say that many people suffer a “drop” when the steriods are initially stopped. I had IV steriods for 3 days and 2 days after they were finished I had to go to bed for 2 days because of exhaustion. I hope that it is the fampyra that has helped as I would like to try that too.

Keep us posted

Moyna x

Hi John,

PPMS is much harder to dx than RRMS and I have read that people have been misdx with PPMS frequently when it has turned out to be something else entirely. My neuro is very reluctant to dx someone with PPMS if they have a clear lumbar puncture. This is also mentioned by the Prof on the Barts MS Blog as he believes MS with a negative LP is an entirely different disease. I dont have a dx of MS as my LP was clear as is my brain scan. I do have a lesion on my cervical cord. I think I fit the PPMS picture better as my walking is the only issue - although the whole progression was started by some sort of attack/relapse nearly 5 years ago.

I was told the postive effects of steriods last 6 months so stopping them for one day should not have effected you. Although I must say that many people suffer a “drop” when the steriods are initially stopped. I had IV steriods for 3 days and 2 days after they were finished I had to go to bed for 2 days because of exhaustion. I hope that it is the fampyra that has helped as I would like to try that too.

Keep us posted

Moyna x