David Systrom, researcher, Brigham and Women's Hospital, USA

Dr. David M. Systrom of Brigham and Women’s Hospital (Boston) is confident that his team has objective evidence of small-fiber polyneuropathy (SFPN) in 40-50% of ME patients.

Dr Systrom was interviewed by Llewelyn King in this video:

https://www.youtube.com/watch?v=FMaKfv8peww

. I put the timestamp at the part involved with SFPN but the whole video is highly informative and worth watching. His specialty is exercise intolerance of any kind and his work lead him to try to differentiate ME patients.

About 40% of fibromyalgia and POTS patients are also suspected to have SFPN and it is a relatively simple test involving a skin biopsy.

The doctor is also confident to have evidence of preload failure, which if I understood and remember half correctly is caused by poor constriction of the lower-body vascular system, leading to decreased ability of the blood to reach the heart. This is tested using an invasive CPET session, with radial and heart catheters.

His team has funding for a double-blind RCT for a drug used to treat myasthenia gravis, pyridostigmine, targeting acetylcholine receptors but possibly also reduces inflammation. Some patients seem to have shown objective benefits from smaller trials. The drug has been approved for decades so it is well-known.

 

Interesting. I've never really paid much attention to "small-fiber neuropathy," considering it to be connected to fibromyaglia, which I assumed was mostly diagnosed by tender points, which I do not have.

However, I have experienced unexplained, and very long-lived sunburn-like sensations on/in the skin of my arms and midsection. Apparently, a burning sensation in the skin is one form paresthesia associated with small-fiber neuropathy. For me, this burning sensation was a lot more noticeable early on, but I still sometimes feel it to this day. When I feel it now, it's clearly less intense than it was originally, probably partly because I've just grown used to it.

 

Excellent video.

 

Welcome to the forum, @rvallee, and thanks very much for posting this very interesting interview. I'm surprised not to have heard more about Dr Systrom's work.

 

https://solvecfs.org/announcing-smcis-new-mecfs-research-program-at-brighams-and-women-hospital/

 

@rvallee

Thanks for posting this - fascinating stuff.

I've been hoping that someone would look into possible SFPN in ME/CFS for a long time as it ticks a lot of boxes as a possible source of ongoing peripheral stimulation that could keep central microglial activation cooking away. Not to mention direct and indirect effects on the autonomic nervous system.

As I noted a while back there's a lot of potential to piggyback on much better funded areas :

Could Cancer Research Improve the Treatment of Widespread pain in Fibromyalgia and ME/CFS?

https://www.healthrising.org/blog/2...treatment-widespread-pain-fibromyalgia-mecfs/

 

I really respect Systrom's work - it couldn't have been easy to convince funders/patients to do invasive CPET studies!! Nice to see him coming across well in these interviews too. I know of a few people that have seen him clinically too, with mixed results (if Pyridostigmine doesn't help you are out of luck).

There was another excellent webinar recently by Systrom that has data and discussion on the studies:
https://www.s4me.info/threads/webin...nal-tuesday-jan-16-7-8pm-est.1917/#post-40954

 

Very interesting. Thanks for posting @rvallee and welcome to S4ME

 

Thanks for the short summary as I can't watch videos well.

I too have the burning sensation of the skin but very little pain. What section does he talk about the skin burning bit

I guess the next step is IVIG.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5791555/

 

Here is what I said on my FB page -

If you have ME and orthostatic intolerance, especially POTS, and any sign of small fiber polyneuropathy, I highly recommend watching this.

They find right heart preload failure, low oxygen use, and over 40% with small fiber polyneuropathy ... but only tested in skin. (My comment is there might be patients with no skin neuropathy, but have neuropathy in internal areas including organs and blood vessels. ) These small fibers are critical in orthostatic regulation. They are working on treatments. They find their patients spend lots of money and 3-5 years getting a diagnosis, and specialists fail to find anything.

They will be launching a double blind RCT using pyridostigmine, otherwise used in myasthenia gravis. This preserves acetylcholine levels. About 300 (mostly) women have improved, and they have objective evidence of improvement, which naturally leads to the need for a clinical trial.

He is looking for multicenter and multidisciplinary research, all communicating across different specialties.

This fits with the hypothesis I have been a fan of for a long time, that many diseases are unsolved while we lack the proper technology.

 

https://twitter.com/user/status/1014186272259223554

 

Dr Systrom seems to think it's a reasonable hypothesis that it could explain the stimuli sensitivity that many of us experience, that one potential mechanism of SFPN is a form of amplified stimulation by lowering the firing threshold. If it can affect pain receptors it's reasonable that it would affect other receptors as well. Could explain the weird temperature regulation.

I think there was another researcher in the Boston area who also found SFPN in ME patients. We'll have to wait for replication and peer review to be more confident but this is good.

I am also excited about this being found in FM and POTS as well. I believe there is a good basis for these diseases to form a new interdisciplinary specialty. It could potentially absorb other disorders as well, like myasthenia gravis. And since SFPN seems to be caused by demyelination, maybe there is overlap with MS, if not every form maybe some of those that do not fit the general MS model.

In Canada, a report from a Ontario health ministry task group estimates that the combination of ME, FM and MCS could be as high as 1M, or 3% of the population. There is a risk/reward to consider about spreading attention away from strictly ME, but I think the potential for increased budget more than makes up for it. It's likely that many of the mechanisms in ME are similar in those diseases and a solution for one of the ME puzzles may come from looking at POTS or FM.

 

Does the nerve conduction test pick up SFPN? I had that with the EMG and the nerve bit was fine for me (muscle abnormal but not specific enough to diagnose). Or is it only skin biopsy?

 

I've been following everything I can find about the disease for 2 years and it's the first I've heard of him. I think there are a lot more people out there working on this than we are aware of. Research cycles are long so that's kind of normal as we only hear when results are published but it's clear that the workload under way is underestimated because no one has the resources to organize it, too many other priorities.

Hopefully this is one of the things the NIH centers will do. If we can miss out on a lot of research despite eagerly looking for them, it's no surprise that most physicians have no idea how much research is happening and how advanced it is.

 

@rvallee

I was surprised and delighted to hear that he had hooked up with Anne Oaklander. I had obliquely suggested that people with Fibro/ME/CFS contact her re SFPN in a previous blog :

https://www.healthrising.org/blog/2...ell-us-chronic-fatigue-syndrome-fibromyalgia/

 

@rvallee,

great video, thanks. Dr Systrom is clear communicator, which always helps. it’s really encouraging to see these practitioners and researchers coming together and trying to push forward understanding and solutions.

@Jenny TipsforME, I think small fiber neuropathy is only diagnosed via the skin biopsy. hopefully, you can find someone experienced with SFN, if you can..

here’s an anecdotal story. 20+ years ago, Long before I had any competent medical advice or knowledge of terminology, I used to call postexertional malaise, “the kickback the next day, etc”. Then I was prescribed Pyridostigmine for other reasons and I swore that it lessened “the kickback”. I stayed using Pyridostigmine for years, but eventually the gastrointestinal side effects just got so horrible that I couldn’t keep using it. It did nothing for neuromuscular pain but it sure seem to work for at least a while on PEM.... i’ve tried going back on a couple times and I just can’t handle the gastrointestinal side effects.

I may try again.

 

Even if the illnesses are distinct entities it would make a lot of sense to cooperate at the science and advocacy level. We're in the same boat, and together we have the numbers to make a difference.

 

While I agree with the rest of your post especially re explaining other 'sensitivities' as far as I can tell the small peripheral nerve fibres are largely unmyelinated so unlikely to be related to MS.

 

here is Dr Systrom's the funding source announcement from last year..

https://solvecfs.org/announcing-smcis-new-mecfs-research-program-at-brighams-and-women-hospital/