Differentiating post-polio syndrome from myalgic encephalomyelitis and chronic fatigue syndrome, 2019, Jason et al

Paywall, https://www.tandfonline.com/doi/abs/10.1080/21641846.2019.1687117
Not available via Scihub at time of posting

 

This is pretty wild. Looking forward to read more on this. Throwing another wrench in the misguided idea that only active infections matter. Especially how it is critical to the BPS model of ME, that an infection may have occurred but once gone its role should be discarded as no longer relevant. Clearly not that simple.

 

I seem to remember the theory of post polio syndrome is that the polio virus damages the end plates of the muscles and as they are used they accumulate damage until after about 30 years people have reached the point where they no longer work properly so it is not a continuing infection.

The treatment is to save as much function as possible with stairlifts, mobility aids, rest, that sort of thing

I have a friend with PPS who is disabled, but not like us, it is more mechanical and less "weird"

 

This abstract looks pretty worthless. They define three things, "PPS", "ME" and "CFS" in the background section. Then, under results, they compared "ME/CFS", which wasn't defined in the background section to PPS. Wow, this journal article must have undergone severe peer review. Sometimes it is helpful if someone at least tries to read these things (at least the abstracts!) before they are published.

Received; 24 Sept
Accepted: 28 Oct
Published: 6 NOv.

 

I had a close neighbour friend who got polio as a child and had a relapse at age 42. She was walking down the street and lost muscle function in her legs and fell down. She eventually recovered, but it was quite a shock for her.

 

https://sci-hub.se/https://doi.org/10.1080/21641846.2019.1687117

 

This is the work of Dr Leonard Jason, a well respected psychologist who happens to do epidemiology as well and who is a patient himself. The type of research he does is not typically blood based, though sometimes he collaborates with scientists on the biological side. His work is very much based on questionnaires and case definitions, and taking a look at post-polio syndrome and comparing it to ME/cfs typical patients is another piece of relevant information, while the post polio patients are still alive. Maybe one day post-Ebola patients will be compared to us as well.

Also Dr Jason is a professor and he works with graduate students.

 

A few studies have found persistent poliovirus infection in the cerebrospinal-fluid and blood of post-polio syndrome (PPS) patients: see this study, this study and this study.

Since poliovirus is an enterovirus, there might be an argument for classifying PPS as another form of enterovirus-associated ME/CFS.

Though the question remains as to why PPS only appears many decades after the acute poliovirus infection. Whereas when ME/CFS is triggered by enteroviruses such as coxsackievirus B, the disease appears within days or months of this acute infection.

One speculative answer might be that chronic poliovirus infection is better controlled by the immune system than chronic coxsackievirus B infection. So it may be only when immune senescence occurs later in life that the immune system starts to have difficulty in controlling poliovirus, and perhaps that's when PPS makes its appearance.

A similar thing happens with varicella zoster virus: it causes chicken pox initially, but then the virus is brought under control by the immune system. But later in life, when the immune system naturally weakens, VZV can reemerge as shingles.

 

Have now read the study.
It's good to carefully compare symptom presentations between superficially similar conditions, both to look for similarities that could point to common affected pathways and to look for differences that could help improve diagnostic accuracy. So this contribution to those efforts is welcome.

This study shows, again, that when compared to another serious condition, ME is the more disabling one (though there's probably a sampling bias in this study).

BUT! But I'm yet again frustrated by the vague descriptors of PEM in the DSQ. The more I see it being used the less I like it.

At first I was actually quite impressed by the DSQ because it seemed to reflect my experience, I could tick all the boxes. But later I realised it really only captures post-exertional fatigue (and pain), not full-blown PEM. And sure enough, many people with other conditions also recognise themselves in the descriptions and also can tick all the boxes, and many with PPS did just that in this study. There's still a significant difference at group level, more "PEM" reported in ME than PPS, but at the individual level there are plenty of people with PPS reporting "PEM".

Which leads me to fear that the DSQ could unintentionally contribute to the watering down of the PEM concept, rather than help strengthen it. There are already BPSers out there claiming PEM is not specific to ME and now they'll undoubtedly say "See, PPS patients get PEM, too, and here's the study to prove it". Thing is we don't know. Maybe some people with PPS do get PEM, ME-style, maybe not. It would be very good to know, in fact, but unfortunately we simply can't tell one way or the other from answers to the DSQ.

Table 5 is interesting in this regard. They basically calculated how good an individual symptom is at distinguishing between ME and PPS. Of the 9 PEM sub-items in the DSQ only 3 feature in the table at all, and only 1 features highly. This would seem to confirm my suspicion that the 9 PEM descriptions aren't all that good.

Anecdote: I used to know someone severely affected by PPS - died from it in fact, so I definitely do not underestimate the awfulness of PPS, but PEM? No. Not this person anyway. Post-exertional fatigue and pain? Absolutely. And bad, too. Tick all the "PEM" boxes on the DSQ? Yes, easily. But PEM as somebody with ME would understand it? Nope.

 

A well written related study: Parallels Between Post-Polio Fatique and [CFS]: A Common Pathophysiology? - Bruno et al 1998

The paralleles are mainly:

• neuropsychologic ones (high normal or superior range of higher level cognitive processes, slowed information processing)
• neuroanatomical ones (hyperintense signals in some brain areas)
• neuroendocrine ones

They invite a Brain Fatique Generator Model (BFG), involving reticular formation (RA, RAS), basal ganglia (BG), thalamic and hypothalamic nuclei, dopaminergic neurons, and some other areas (sometimes able to get connected to special symptoms).

from the Conclusion

I don´t see any problems to localize PEM in these structures, rather the other way ´round: These structures will especially well explain the wide range of PEM symptoms (or of PEF), and even more strikingly delayed PEM (as it would be an effect of information processing over the course of time). (@Ravn)

I would disagree with this article in its preferred interpretation that there would be a tissue damage by viruses, or even a tissue alteration at all (though well possible, I would say, even in both directions). As far as I see for now, the article does not assume that there is an ongoing infection (they also say "polio survivors").

This pretty intelligent article is already from 1998. Hardly to believe.

 

My impression based on Dr. Leonard Jason'd past questionnaires is that he did not fully understand PEM very well.

 

I have been thinking about how to describe my PEM or give a description of what happens when I exert myself. We just do not have the right vocabulary; every time you try to put it into words it sounds like something different that people with other diseases might get.

I have decided that anyone who claims that other diseases get post exertional malaise does not understand what it is. One of the reasons we find it so hard to talk about is that it is so different from anything else we experience in life or that anyone outside the ME community describe.

A unique response to exertion, that is what PEM is.

 

Yes, Mithriel, it is different from anything else patients experience in life. My daughter says this too; when I pester her to liken it to something I might have experienced, she says she simply cannot compare it to anything she ever had when she was well and normal. She says she cannot compare it to any of the pains in various parts of the body she had as a well person.

 

I came across this independently and found it interesting. There are strong parallels with post-polio fatigue and CFS fatigue, including PEM (though I know some people don't want to hear that)...

edit - a more readable link to the aforementioned manuscript:
https://www.papolionetwork.org/uplo...en_post-polio_fatigue_and_chronic_fatigue.pdf

 

Personally, I see no logical reason currently why polio couldn't be the cause of ME for someone. Or why it couldn't be one of many immune challenges that, in a susceptible person, leads them to develop ME.

 

Interesting that they present Type I, Type II and Type III poliovirus.

I think this will also be the case for M.E.

 

Type I, II, and III polio are just names for different enteroviruses. They are all very similar but these are the ones which were included in the polio vaccine as they were the commonest cause of the epidemics. Enteroviruses which have different names, rather than just numbers are just an accident of history.

The closeness of symptoms of ME and post polio syndrome may indicate that a lot of us have an enteroviral cause (it was always believed that subclinical infections could cause ME so some people may not realise they had an infection) It matches a lot of my problems and my illness began with a Coxsackie B infection.

However, PEM is not part of post polio. There is no delay in experiencing symptoms after overdoing things and certainly none of the thinking you had kept within your limits and then collapsing 3 days later which so many people have. I don't think there is the immune symptoms we get either; the swollen lymph glands, sore throats and so on.

Even if it is only a subgroup I wish more people were looking at enteroviruses and ME.

 

I don't experience a delay either, but I do experience worsening due to PEM. Perhaps I don't have ME...

Post-polio patients can experience swollen lymphs and so on, albeit they report them with less frequency than ME patients. Likewise, those with severe fatigue post-Guillain Barre Syndrome also experience these sorts of symptoms along with PEM-like experiences.

 

I have been thinking about this, what exactly is ME and how does it relate to other enteroviral infections. I am probably one of the few people on the forum who experienced a polio epidemic so grew up with people who had the braces and other problems.

Now with post polio syndrome, they do not seem like ME patients in some ways but very alike in others.

Expertise in enteroviral infection was lost with the success of vaccination and the passing of time (like with TB) and these bigs are often dismissed as trivial things that everyone gets. Yet they can have devastating consequences for the brain and nervous systems which could account for many of the "medically unexplained" functional neurological disorders.

I wonder if the dauer (?) they found is actually a protective response when the body is so damaged it is necessary to induce fatigue to allow the body to survive. Cyclists have died because they take cocaine to prevent fatigue but they then overexert.

That fatigue may not cut in until the aerobic system has been damaged in some cases which could account for the many different ways that ME seems to start, but then a damaged respiration system could also be one of the reasons the body goes into protective mode.

Enteroviral ME where the brain is damaged could account for the weirder symptoms some people get.

When CFS was invented the US researchers concentrated on EBV and the immune system whereas the UK researchers had been concentrating on enteroviruses and abnormal energy production. I hope that more work is finally done on that.