Periodic Paralysis, myotonia, channelopathy and ME

[Gingergrrl]

How are channelopathies examined?

This is a great question and even though I have been diagnosed with a calcium channelopathy, I do not know the answer and do not think that any doctors do either (except maybe Dr. Angela Vincent in the UK but I have given up that I will ever be able to reach her).

If a person with PP would supplement potassium (or sodium in any form), what would happen?

This is a great question, too, and in my case, I had the same questions re: calcium and re: meds and supplements that block the calcium channel (like Magnesium). Neuros do not agree b/c they do not know. I think medicine will eventually catch up but not sure it will be in my lifetime. The ultimate consensus for me was not to take meds that further block the CA channel (although I do okay with a small amount of Mg Malate in pill form).

Yes but often simply due to lack of funding for decent research to test them

I agree and just b/c of lack of funding it does not mean that these disorders are not real or should not be taken seriously.

I genuinely didn’t see that before posting here

I think it is okay to post in both places b/c each board has different members with different areas of expertise.

Ppl are still definitely talking about channelopathies and autoimmunity to neuron-associated receptors like the muscarinic cholinergic, hasn't fallen off the map so far as I'm aware.

@JaimeS This really describes my situation (channelopathy, autoimmunity, and antibodies to the muscarinic/cholinergic receptors) and I am glad to hear that this research has not fallen off the map. I just wish I understood more about it and if there were other treatments that I could be doing (even though I feel that I am on the right track with what is known at this time in medicine).

When I went to Open Medicine Institute, and mentioned paralysis, they immediately thought Myasthenia Gravis. It took over a year to get approval from my HMO for MG testing (including calcium channel), which came back negative. I did a trial of Mestinon, but it didn't help.

Everyone always thinks of MG first, which makes sense b/c it is more common, well-known, and accepted by modern medicine. I was also tested for MG and MuSK auto-antibodies and they were all negative. My understanding (and I hope I do not screw this up!) is that the MG auto-antibodies affect the nicotinic receptors vs. the other ones affect the muscarinic receptors (but only the MG/nicotinic ones are tested for in the US) which is so odd to me. Hoping Jaime can correct me if I got that wrong.

So right now I have a diagnosis of "Myasthenia" but not Myasthenia Gravis. According to my neurologist, Myasthenia Gravis has not been completely ruled out yet, however.

How are they treating your diagnosis of "Myasthenia" (or are they not treating it since the Mestinon did not work)?

All because there is no published study, does not mean that a hypotheses is true or false. It takes a lot of money to perform a study, and not everyone with a hypothesis can afford to go this route.

ITA and without money to fund these studies, so much good research will be lost forever.

 

[Webdog]

How are they treating your diagnosis of "Myasthenia" (or are they not treating it since the Mestinon did not work)?

Not treated.

 

[Gingergrrl]

Not treated.

Why am I not surprised?

 

[merylg]

https://newsroom.uw.edu/news/study-reveals-why-rare-disorder-causes-sudden-paralysis

 

[Jenny TipsforME]

I have some interesting stuff to add to this thread but too foggy right now.

 

[Inara]

https://newsroom.uw.edu/news/study-reveals-why-rare-disorder-causes-sudden-paralysis

Very interesting!

 

[Sly Saint]

Haven't read through this thread but was reading a bit about Neuromyotonia
https://en.wikipedia.org/wiki/Neuromyotonia

and spotted this:
"Some neuromyotonia cases do not only improve after plasma exchange but they may also have antibodies in their serum samples against voltage-gated potassium channels.[3] Moreover, these antibodies have been demonstrated to reduce potassium channel function in neuronal cell lines."
"It is speculated that the plasma exchange causes an interference with the function of the voltage-dependent potassium channels,"

could this relate to Ron Davis research?

@Ben H

 

[daftasabrush]

Moderator note: This post and several posts discussing it have been moved from this thread:
https://www.s4me.info/threads/trial...re-sharpe-and-chalder.9107/page-2#post-160657

You need to add Functional Neurological Disorder (FND) to the long list of what to look out for. A lot of patients are being re-diagnosed with it. The mail Yesterday did a piece on a young girl with head injury that was diagnosed with it once and recovered with CBT. Unfortunately she had another blow to the head and suffer more problems.

As far as I'm aware FND is under dissociative neurological symptoms disorder in ICD-11


https://www.dailymail.co.uk/health/...ft-18-kayaking-world-champion-wheelchair.htmlView attachment 6885

Ironically, the FND comparison of a computer with software that does not work actually DOES fit a number of physical conditions, including hypokalemic periodic paralysis and normakalemic periodic paralysis - with HypoPP being found in some with ME, as described in Stonebird (acquired form), and from rare diseases search http://findzebra.com - it feels exactly like your brain can't communicate with parts of your body for a while.
Triggers include high carbohydrate meals, over-exertion and temperature changes eg cold.

In Focus: It’s All in the Muscles, Not the Head. Muscular Dystrophy Association

The cause of hypokalemic periodic paralysis? Ion transportation issues (channelopathy involving the sodium channel) - this imbalance means communication cannot be transmitted between cells, eg in the spinal cord or muscles, leading to episodes of paralysis - basically no permanent physical damage, no signs when not having an attack, just like the software metaphor without the baseless psychologization.
Ion transportation symptoms are described in the ME International Consensus Criteria as diagnostic criteria! Treatment? Restore electrolyte balance.

 

[daftasabrush]

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?

Or do lots of us have a comorbidity that goes undiagnosed?

Or is PP properly separate but ME and POTS are more common so a likely misdiagnosis?

There's an acquired form

 

[daftasabrush]

Thank you, @duncan!

If a person with PP would supplement potassium (or sodium in any form), what would happen?

supplement with potassium for hypokalemic periodic paralysis or nomakelmic, less potassium for hyperkalic form

 

[daftasabrush]

ME-ICC diagnostic criteria
D. Energy Metabolism/Ion Transportation Impairments: At least One Symptom
1. Cardiovascular: e.g. inability to tolerate an upright position - orthostatic intolerance (OI), neurally mediated
hypotension (NMH), postural orthostatic tachycardia syndrome (POTS), palpitations with or without cardiac
arrhythmias, light-headedness/dizziness
2. Respiratory:e.g.airhunger, laboured breathing, fatigue of chest wallmuscles
3. Lossof thermostatic stability:e.g.subnormalbodytemperature,marked diurnal fluctuations; sweating episodes,
recurrent feelings of feverishness with or without low grade fever, cold extremities
4. Intolerance of extremes of temperature

Candidian Consensus Criteria - recognized paralysis but decided it was not not common enough to include as diagnostic criteria. Based on a study of 2,500 people with "chronic fatigue" who were then divided as per Fukuda, with additional symptoms added to rule out cases with other causes of chronic fatigue.

Acquired periodic paralysis - triggers include over-exertion, high carbohydrate meal (for some types), hot or cold temperatures, and others

Great resource for triggers - skip past the genetic stuff Periodic Paralysis. In Focus: It’s All in the Muscles, Not the Head -
Muscular Dystrophy Association

See also Linda's experience and Greg's paralysis survey and channelopathy article - Stonebird - Greg's mention of the Parkinson-like "getting stuck" too.

All this is regarding ME triggering the condition - not having HypoPP rather than ME

http://me-pedia.org/Hypokalemic periodic_paralysis

 

[James Morris-Lent]

The hardware/software analogy has its limits but a channelopathy would generally qualify as dodgy hardware.

 

[John Mac]

The BPS lot believe you can cure hardware problems by rewriting the software, even when the evidence points to a problem in the power supply.

 

[Arnie Pye]

hypokalemic periodic paralysis

So, why don't doctors treat this in the short term with potassium supplements, and give the patient dietary advice on which foods they can eat to raise their potassium levels and prevent the problem from happening in the first place? Or is this condition more complicated that it appears on the surface?

 

[daftasabrush]

You need to add Functional Neurological Disorder (FND) to the long list of what to look out for. A lot of patients are being re-diagnosed with it. The mail Yesterday did a piece on a young girl with head injury that was diagnosed with it once and recovered with CBT. Unfortunately she had another blow to the head and suffer more problems.

As far as I'm aware FND is under dissociative neurological symptoms disorder in ICD-11


https://www.dailymail.co.uk/health/...ft-18-kayaking-world-champion-wheelchair.htmlView attachment 6885

Ironically, the FND comparison of a computer with software that does not work actually DOES fit a number of physical conditions, including hypokalemic periodic paralysis and normakalemic periodic paralysis - with HypoPP being found in some with ME, as described in Stonebird (acquired form), and from rare diseases search http://findzebra.com

https://www.mda.org/sites/default/files/In_Focus_Periodic_Paralysis.pdf Periodic Paralysis.
In Focus: It’s All in the Muscles, Not the Head
Margaret Wahl 2009
Muscular Dystrophy Association


The cause of hypokalemic periodic paralysis? Ion transportation issues (channelopathy involving the sodium channel) - this imbalance means communication cannot be transmitted between cells, eg in the spinal cord, leading to episodes of paralysis - basically no permanent physical damage, no signs when not having an attack, just like the software metaphor without the baseless psychologization.
Ion transportation symptoms are described in the ME International Consensus Criteria as diagnostic criteria! Treatment? Restore electrolyte balance.

So, why don't doctors treat this in the short term with potassium supplements, and give the patient dietary advice on which foods they can eat to raise their potassium levels and prevent the problem from happening in the first place? Or is this condition more complicated that it appears on the surface?

Actually it really is that simple - lifestyle advice with potassium. It's a rare illness so often missed.

Also fits with Dr Myhill's low carb advice (although not everyone needs low carbs).

In severe cases, IV potassium mix.

Good example here

 

[duncan]

Actually it really is that simple - lifestyle advice with potassium.

Few things in our orbit are that simple, including most cases of channelopathies that I am familiar with. Potassium adjustments through diet can help mitigate episodes, but not many sufferers I am familiar with are cured.

 

[Hoopoe]

I'm not familiar with this topic, but is this the correct usage of the term paralysis? The patient is still moving his limbs, although he seems extremely fatigued.

This is a bit similar to what I have occasionally which is extreme fatigue leading to collapsing on the bed in a semi-awake state where I have no desire to move any part of my body. It seems to be brought on by eating a lot of carbs.

 

[duncan]

I'm not familiar with this topic, but is this the correct usage of the term paralysis? The patient is still moving his limbs, although he seems extremely fatigued.

It is the correct label. Its accuracy is similar to CFS in that some people have fatigue as a principal complaint in CFS, some do not. In Periodic Paralysis, some people are paralyzed sometimes, some people never are, but suffer profound weakness in their muscles, or have other issues.

If you have ME/CFS and you want to see what other patients or experts have to say, you go to an ME/CFS forum. If you have a channelopathy, odds are you go to a Periodic Paralysis forum.

I suspect there are a comparable amount of PP experts to ME/CFS experts, ie, not many.

 

[daftasabrush]

The BPS lot believe you can cure hardware problems by rewriting the software, even when the evidence points to a problem in the power supply.

would love to see this explanation in a meme

BPS = doing the same thing repeatedly (since the 1990s!) and expecting different results - insanity - it didn't work then, it doesn't work now - even null trial results by BPS-ers like the FINE Trial don't discourage them

 

[Jonathan Edwards]

I'm not familiar with this topic, but is this the correct usage of the term paralysis? The patient is still moving his limbs, although he seems extremely fatigued.

From what I can gather this person has not got a diagnosis of channelopathy. It is just a U-Tube video of someone with episodes of subjective weakness - whatever the cause might be.