In response to the previous comment from the MS’ers with PPMS, who needed a walking aid within 4years of disease onset and malignant MS.
The following is the survival curve in relation to PPMS. As you can see only ~8% of MS’ers with PPMS need a walking stick within 4 years (red); this is twice the progression rate of the average, i.e. 50% require a walking aid within 8 years (blue).
This study reports the natural history of 216 MS’ers with PPMS defined by at least 1 year of attack-free progression at onset. This represents 19.8% of a largely population-based patient cohort having a average follow-up of 23 years.
This subgroup of PP-multiple sclerosis patients had a average age of onset of 38.5 years, with females predominating by a ratio of 1.3:1.0.
Disease course:
- The rate of deterioration from disease onset was substantially more rapid than for RRMS, with a median time to EDSS 6 (cane) and EDSS 8 (restricted mobility) of 8 and 18 years, respectively
- EDSS 6.0 = Intermittent or unilateral constant assistance (cane, crutch or brace) required to walk 100 meters with or without resting.
- EDSS 8.0 = Essentially restricted to bed, chair, or wheelchair, but may be out of bed much of day; retains self care functions, generally effective use of arms.
Examination of the early disease course revealed two groups with adverse outcomes:
- A shorter time to reach EDSS 3 (moderate disability, though fully ambulatory) from onset adversely influenced time to EDSS 8.
- Involvement of three or more neurological systems at onset resulted in a median time to EDSS 10 (death) of 13.5 years in contrast to PPMS patients with one system involved at onset where median time to death from MS was 33.2 years.
- Age, gender and type of neurological system involved at onset appeared to have little influence on prognosis.
Life expectancy:
- Cause of death and familial history profile were similar in PPMS and relapse-onset MS.
PPMS vs. SPMS:
- From clinical onset, rate of progression was faster in the PPMS than in the SPMS.
- However, when the rates of progression from onset of the progressive phase were compared, SPMS had a more rapid progressive phase.
Relapses:
- A substantial minority (28%) of the PPMS’ers had a distinct relapse even decades after onset of progressive deterioration.
“Apologies, if some of you find these figures grim reading. Unfortunately, as you know, MS is a bad disease, particularly PPMS, and these figures make that very clear. This why we are spending a large amount of time working on progressive disease; how to stop or slow it down or in the case of relapse-onset MS prevent it from starting in the first place.”
Additional reading: EDSS
This is a totally depressing post. It just proves how PPMS is the worst of a bad bunch and that there is nothing anyone can do to stop it. Worst of all, I’ve never had the science of PPMS explained to me. No one even really knows what’s happening in the nervous system. My neurologist simply says he’s not sure exactly what the specifics of PPMS are. This guy is meant to be a NHS neurologist! Jesus Christ, there are some hopeless people working in the field of multiple sclerosis.
Re: "I’ve never had the science of PPMS explained to me."I am pretty sure that PPMS is not a different disease to relapsing MS. I suspect that PPMS'ers miss out on the relapsing phase as they don't have any lesions in eloquent areas. It is important to note that PPMS starts at exactly the same age as the SP phase of the disease and ~20% of PPMS'ers will go onto to have superimposed relapses. A large number of people state that PPMS is not associated with inflammation, however, this is not borne out by pathological studies. I therefore believe that if we are to impact on PPMS we need to target both inflammation and the neurodegenerative processes, i.e. using a combination therapy. The good news is that there are currently two trials looking at anti-inflammatory therapies in PPMS; fingolimod and ocrelizumab. In addition, there are some interesting neuroprotective therapies being explored. So it is not all bad news.
Do you think that people dx with PPMS simply don't realise that they have had 'episodes' earlier in life and are therefore SPMS? Or is there something distinctive about PPMS that you are able to use to aid diagnosis? I presume the latter is the case but find myself wondering about the former.
Re: "Do you think that people dx with PPMS simply don't realise that they have had 'episodes' earlier in life and are therefore SPMS?"Yes, for some this is clearly what happens. When you go back into the history in detail you often find symptoms that could have been due to an attack (or attacks). This is often referred to as a sentinel attack. At one point in time Professor Alan Thompson referred to this as being transitional MS; this term never gained wide acceptance. For the majority, however, it is simply the slow insidious onset of neurological symptoms without any attacks that characterises the course.
If it's the same disease, why are people with PPMS left out of clinical trials and why don't they get/respond to the same treatments?
Re: "If it's the same disease, why are people with PPMS left out of clinical trials and why don't they get/respond to the same treatments?"It is the same disease, but similar to SPMS. Unfortunately this phase of the disease has proven difficult to modify. Most treatments target relapses, which are easier to measure. We still have difficult doing progressive trials; our trial designs are poor and we need to many MS'ers to be followed for too long a time to get answers. This is why we are trying to promote new trial designs to get quicker answers on fewer patients. Would you be prepared to have 3 lumbar punctures as part of a trial to test neuroprotective therapies?
I asked on behalf of somebody else, but yes, any sensible person would accept 3 or more lumbar punctures. I don't think you need to worry about that.
What would you class someone who's gone from no symptoms to spasticity in all limbs, sensory issues in all and urinary urgency? Is this malignant Ms? Marburgs or something else
My son 46 died in bed last week he was diagnosed with progressive MS and on two occasions passed out. I believe he was given steroid injections recently. The coroner is continuing the post Mortem which will take five days but so far they haven't any results. What do you think would have caused his death?