Periodic Paralysis, myotonia, channelopathy and ME

Jenny TipsforME

Jenny TipsforME

Senior Member (Voting Rights)
My experiences, which I’ve discussed at length on The Other Forum, have led me to read up a bit about Periodic Paralysis.

Reading more I feel like it might well have some biological connection to ME and/or POTS. The name is slightly misleading, it does involve symptoms other than paralysis which can include brainfog, phases of weakness and delayed reaction to exercise. They seem to have something like PEM though there are variations in how it expresses.

If you read this account, she could easily have been misdiagnosed with ME:

Imagine yourself hanging out with friends, shopping at the local mall. All of a sudden, for no reason that you are aware of, your legs become so heavy that you can no longer pick them up to take another step. Your friends think you are joking, but you're not.

Or perhaps, picture yourself at a birthday pool party. Surrounded by family excitement, the summer sun, and plenty of junk food, you begin to experience severe dizziness accompanied by debilitating weakness in your entire body. Your family is baffled by your sudden impairment and have no idea why you're being so dramatic or what to do to help.

Last but not least, let's say you are in a well air-conditioned home, washing dishes at the kitchen sink. Within five minutes, your limbs begin to cramp and go numb. You stop what you're doing and barely make it to your bedroom before collapsing into bed. After a power nap spawned by pure exhaustion, you wake up and cannot move your body at all. With no other choice, you lay there until you are able to get up...however long that may be.
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http://fightinghkpp.blogspot.co.uk/2012/05/the-battle-against-periodic-paralysis.html

I’ve definitely had very similar experiences, as well as more full on paralysis. Although my stamina is very limited, what most intrigues medics is my changing strength. I can have an abnormal neuro exam and EMG showing proximal weakness, then another day I can have a normal neuro exam. I can walk up and down stairs like a healthy, then an hour later not be able to walk a step.

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The Gale Encyclopedia of Neurological Disorders describes the Periodic Paralyses as "several rare, inherited muscle disorders marked by temporary weakness" (656). According to Periodic Paralysis International, these episodes can last anywhere from a few minutes to many days, and repeated periods of weakness can eventually result in loss of overall muscle strength (hkpp.org). It is an autosomal dominant ion channelopathy, which means the condition passes down from parent to child in most cases, however some patients' family history is undetermined (hkpp.org).
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Periodic Paralysis is a very rare condition and it is properly ‘genetic’, involving specific mutations rather than a collection of genetic tendencies. It is to do with potassium/sodium/calcium channel problems.

I do wonder though if it is one of those conditions that is rarely diagnosed rather than genuinely so rare. Or it might not be so rare for us. In the same way that POTS goes from unlikely to 1/3 probable if you have ME, there might be some reason for comorbidity.

Also people with PP have told me they’ve noticed comorbidity of POTS, so that seems to be a thing (even if it only applies to the POTS&ME subgroup).

In terms of illustrating what I’m wondering:

Is there a small comorbidity like this?

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Or do lots of us have a comorbidity that goes undiagnosed?
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Or is PP properly separate but ME and POTS are more common so a likely misdiagnosis?
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If you’re curious to find out more here’s a booklet about diagnosis: https://docs.google.com/open?id=0B-savOlQA6QCZjRjODdlMWYtM2M4NC00ODFlLWE4MDMtMGIyOWFmZjJiMmNi

A common misperception that doctors have is potassium levels are abnormally high or low (for the general population) during episodes. It isn’t necessarily that obvious, it’s about reacting badly to the change in your body. So someone with hypokalemic periodic paralysis may have technically ‘normal’ potassium levels both during episodes and out of them, but during an episode of paralysis (or other symptoms) their potassium will have dropped relative to when they are asymptomatic.
 
@Jenny TipsforME , my wife has PP, and I also see striking similarities between many of her symptoms and those of other PP patients with symptoms of pwME.

BTW, there is a special PP patient association through Periodic Paralysis International. My wife is part of it, and the info she gets is very helpful since there is a dearth of data on PP - just as there is with ME/CFS. It's an email chain, and it is heavily vetted, but if you are not familiar with it, it may be worth looking into it.

Yes, PP comes with payback for doing certain things, and also for consuming the wrong things. Indeed, part of the diagnostic process involves provoking an episode to discover whether a given patient is hypo or hyper.

And yes, this most certainly is a neurological topic. :)
 
There was another thread on PR and the idea of a sodium channelopathy came up briefly because of something to do with sodium...I think it had something to do with Davis?...I'm sorry, my memory...But like you I too have wondered if somehow at least a subset of pwME triggered a channelopathy. People have acquired channelopathies, so it's not a crazy idea. I suspect no one is looking: very few are looking at PP and very few are looking at ME/CFS, so the odds of someone seriously exploring this as an option likely are remote.
 
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That’s the reason I started this thread @duncan my hunch is there’s something to be found here and no one is looking in the right place.

My PR thread was looking for personal answers, but with this thread I’d like to explore the general idea of a connection between periodic Paralysis, ME and POTS. I do think POTS is relevant here. I don’t have the expertise to work out why, but I don’t think the apparent comorbidity and sodium/potassium balance overlap is coincidental.
 
Most neurologists don't know anything about PP even though it falls within their domain. How many neurologists are competent about ME/CFS? The only "expert" I can think of that is a neurologist would be Natelson.

Even when it comes to POTS, most of us get referred to an electrophysiologist instead of a neurologist.

I agree with your premise, @Jenny TipsforME , but I wonder who would have the expertise to test?

I suppose it would be a pwME cohort examined by a PP expert, but if a channelopathy response cannot be provoked - what then? If PEM can be triggered, a sodium or potassium imbalance may not occur. But it would be cool to find out...
 
This is interesting to me as I have had a recurring pattern of what I would term 'mini strokes' or periodic ataxia when I overdo things.

It's not paralysis per se but it's always the same - my right foot starts sticking out, then the whole leg starts dragging, then both legs. I end up unable to do more than shuffle and only by concentrating, unable to lift my head think or speak. Usually a few hours in bed helps but on occasion it's taken weeks to get back to my 'normal'.

Presumably as an inherited disorder periodic paralysis (the symptom) is a lifetime pattern rather than acquired?
 
@Marco , PP can be inherited or acquired, from what I understand. Symptoms can appear early in life or later, even when inherited. My wife was born with certain PP characteristics, but most of the more serious ones appeared after she was an adult.

Also, even though it is referred to as Periodic Paralysis, some (many?) sufferers never experience paralysis - just profound weakness (along with a bevy of other symptoms).
 
@Marco actually no it isn’t necessarily lifelong, though it’s unusual to get past your mid twenties without at least an episode of weakness (some people with the mutations never get full on paralysis, but they know because of family testing).

Interestingly advice is to not disregard weakness which was labelled CFS when thinking back to when it started.
 
@Jenny TipsforME , there are not a whole lot of competent PP experts out there, and even less with any budget. Do you know when the last ATS research by the NIH was performed? YEARS ago.

But I agree - that's likely the way to go. That private email thing that Periodic Paralysis International provides touches on some of the few good specialists from time to time. Those patients are like us - they take research into their own hands as much as they can.
 
I’m not the first to think of this apparently

The symptoms of chronic fatigue syndrome are related to abnormal ion channel function

A. Chaudhuri
,
W.S. Watson
,
J. Pearn
,
P.O. Behan

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The pathogenesis of chronic fatigue syndrome (CFS) is unknown but one of the most characteristic features of the illness is fluctuation in symptoms which can be induced by physical and/or mental stress. Other conditions in which fluctuating fatigue occurs are caused by abnormal ion channels in the cell membrane. These include genetically determined channelopathies, e.g. hypokalemic periodic paralysis, episodic ataxia type 2 and acquired conditions such as neuromyotonia, myasthenic syndromes, multiple sclerosis and inflammatory demyelinating polyneuropathies. Our hypothesis is that abnormal ion channel function underlies the symptoms of CFS and this is supported also by the finding of abnormal cardiac-thallium201SPECT scans in CFS, similar to that found in syndrome X, another disorder of ion channels. CFS and syndrome X can have identical clinical symptoms. CFS may begin after exposure to specific toxins which are known to produce abnormal sodium ion channels. Finally, in CFS, increased resting energy expenditure (REE) occurs, a state influenced by transmembrane ion transport. The hypothesis that ion channels are abnormal in CFS may help to explain the fluctuating fatigue and other symptoms.
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I only had "paralyses" two times, both in the right leg. I imagined it to be "exhaustion symptoms". I think there could be several reasons, and it's nothing like you report here.
 
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