Poll: Have you ever experienced complete paralysis of a part of your body?

[Pyrrhus]

Has anyone heard of Andersen-Tawil syndrome?

https://twitter.com/i/web/status/1454465142738010115

 

[Inara]

It won't be a surprise Yes.

Why does it interest you?

 

[Jenny TipsforME]

This is a weird experience for me - I saw this poll on Twitter but I’m the person who started the previous discussion linked in the first post here. I’ve not been up to keeping up with forum discussions. The poll doesn’t exactly fit for me, I ticked non genetic diagnosis but it was a bit unclear how they described it.

In my experience it’s better with medics to assert that this type of paralysis (which goes fairly quickly) doesn’t fit ME. Of course that’s more related to the neglect we experience as pwme - ME symptoms are dismissed - but my hunch is it’s a comorbidity at least.

I seem to get 2 types of paralysis, which confuses explaining it to doctors. Since having covid it’s become clearer that I get a POTS related orthostatic trigger (guessing what people on the other thread described as a POTS stroke). I get this even sitting upright and it comes on very quickly so I can’t move at all. It seems to be an interaction of not enough blood getting to my brain to send out signals to move, but the POTS response prevents a simple faint. I’m stuck in the position I’m in, often unable to speak or speaking gibberish. It’s scary and I don’t know what would happen if I’m stuck like that home alone. POTS drugs especially fludrocortisone and mestinon really help but aren’t a miracle cure (mestinon benefit suggests it’s acetylcholine related too?).

I also seem to get paralysis which is more like Primary periodic paralysis but perhaps caused by insufficient ATP. It can come on lying down, it’s slower to start and I get prodrome sensations. When I had proper testing for PPP my results were borderline though and because my symptoms are severe the team was unconvinced this is the relevant diagnosis (but perhaps just confused by the POTS aspects? Eg they reckoned you don’t lose speech, but now I think it’s the orthostatic triggered episodes when I lose speech). The ATP related genetic Atypical Periodic Paralysis was up in the air re my specific SNPs .

 

[Jenny TipsforME]

BTW I’m not sure if the term ‘complete paralysis’ is helpful in differentiating Primary Periodic Paralysis from ME - it appears this can be experienced as variations of weakness severity (and as I described the UK periodic paralysis experts don’t expect you to lose speech). The main thing that would differentiate it from mainstream ME weakness is the way it goes away again. Usually if you’re that sick with ME the severity level lasts a long time, even if the symptoms change. You don’t get Very Severe and then back to Moderate in hours.

There are several overlaps between ME and Periodic Paralysis, though ME is much more likely a diagnosis

https://twitter.com/i/web/status/1078600220668280832

 

[Jenny TipsforME]

I think what I’m describing as orthostatic triggered episodes are what you mean by complete paralysis though - I can’t even move my fingers and it’s like the neurotransmitter signal to move doesn’t get through.

For the other type it feels like there’s something out of balance causing profound weakness, which my body takes a few hours to correct. The message does get to my muscles but my muscles are unable to respond.

 

[Inara]

Cool @Jenny TipsforME that you replied after all these years.

Re. POTS as trigger:
Epinephrine activates the Na-Ka-pump, which often leads to the potassium dysbalance observed in PP. Or: epinehrine often is a paralysis trigger in hypoPP.
I not sure how it is with norepinephrine, but I guess both often come hand in hand, or that there is some other connection.

I have neuropathic and hyperadrenergic POTS. I had to do a Schellong test once, and directly afterwards I developed a paralysis attack. I wondered why. My speculation is: In hyperadrenergic POTS too much norepinephrine - maybe epinephrine, too? - is released. Epinephrine activates Na-K-ATPase, which leads to potassium shifts etc.
The paralysis is a result of hypoPP, not POTS. Someone without PP wouldn't get a problem with the resulting potassium shift. But the POTS is a trigger.

I have observed that POTS is often a comorbidity of PP - which also did Dysautonomia International, and, then, they wanted to do some research on that.

I also seem to get paralysis which is more like Primary periodic paralysis but perhaps caused by insufficient ATP. It can come on lying down

Rest is a well-known trigger in hypoPP. Short: During activity/exercise, potassium rises, afterwards it decreases. That's normal. In hypoPP the decrease can lead to a paralysis attack.
In PP it's about potassium balance between inside and outside the cell, and symptoms occurr much earlier than in normals.

When I had proper testing for PPP my results were borderline though and because my symptoms are severe the team was unconvinced this is the relevant diagnosis (but perhaps just confused by the POTS aspects? Eg they reckoned you don’t lose speech, but now I think it’s the orthostatic triggered episodes when I lose speech)

There are doctors out there claiming to be PP experts while they're not... One PP "expert" claimed I faked, another that it's hyperventilation (a neurologist said hyperventilation doesn't cause paralytic attacks, and another one none that can be resolved with potassium). Many so-called "experts" have textbook knowledge about PP, and this usually does not describe reality. These doctors also don't listen to patients, i.e. they are not willing to find objective reality. Thus, they are worthless.

During an attack, I can't properly speak. In fact it sounds like what you are describing.

 

[Inara]

and as I described the UK periodic paralysis experts don’t expect you to lose speech

In UK, there are Hanna et al. who do some PP research, but they were not recommended to me by people who know better. I.e. it sounds very much like this team is "textbook PP experts".

 

[Jenny TipsforME]

In UK, there are Hanna et al. who do some PP research, but they were not recommended to me by people who know better. I.e. it sounds very much like this team is "textbook PP experts".

Yes it was that team but not Hanna himself. It was a frustrating appointment. I’ve definitely heard other people with Periodic Paralysis say they can’t speak during an episode.

Who would you see in the UK? The trouble is now I probably wouldn’t risk travelling to an appointment again. I had bad PEM for a couple of months after the London appointment and it didn’t achieve anything apart from frustrating me. I didn’t really even get a clear answer that it wasn’t PPP, though they felt it was more ‘faulty wiring’.

I don’t remember how much you followed the previous thread but a geneticist thought the ATP related form of Atypical Periodic Paralysis could fit my whole genome results. A twist in that was a potential lab error, I retested but I’ve not been well enough to go through all the genetic stuff again. My hunch/home testing indicates it isn’t potassium related PP though for me.

 

[Pyrrhus]

@RivkaRivka mentions a relevant paper by Leonard Jason:

Assessment of Post-Exertional Malaise (PEM) in Patients with Myalgic Encephalomyelitis (ME) and Chronic Fatigue Syndrome (CFS): A Patient-Driven Survey (Holtzman et al., 2019)
https://www.ncbi.nlm.nih.gov/labs/pmc/articles/PMC6468435/pdf/diagnostics-09-00026.pdf

https://twitter.com/i/web/status/1460264880641126406

 

[Jenny TipsforME]

I have observed that POTS is often a comorbidity of PP

Yes I’ve observed this in periodic paralysis groups too. Obviously PP is rare so not many people with POTS have PP. But if you have PP the chances of having POTS seems quite high.

So an interaction makes sense.

 

[Inara]

Who would you see in the UK?

Maybe nobody. I would maybe try to get to Dr. W. in Germany. (Yeah, but... I know.) I would contact the PPA for help. E.g. they have "Ask the Experts" and other things... They are very supportive.

 

[Pyrrhus]

A blog from Greg Crowhurst:
http://carersfight.blogspot.com/2019/01/beyond-catastrophic-paralysis-in-severe.html

Excerpt:

BEYOND CATASTROPHIC : PARALYSIS IN SEVERE ME

January 20, 2019

If I make an unexpected noise or movement, no matter how slight, or cause any kind of unexpected
shock, the impact upon my wife, who has Very Severe ME is way beyond catastrophic.

In the diagram below we have tried to show the possible triggers : just about anything!


It is the greatest possible pain to see my wife's whole day totally destroyed by something apparently small and insignificant that I have done; the hour upon hour - if not days of intense naked suffering that follow.
YOU HAVE TO ULTRA AWARE AT ALL TIMES, as carer!

As the diagram shows, it is not just my actions. Sleep, rest, sitting are also common triggers.

Yet, paralysis, which was frequently mentioned by the early authors, especially Ramsay, is rarely if ever mentioned today by clinicians...

 

[Pyrrhus]

A blog from Greg Crowhurst:
http://carersfight.blogspot.com/2019/01/beyond-catastrophic-paralysis-in-severe.html

Related discussion:

Crowhurst: Paralysis Study - 46 Patient Responses
https://forums.phoenixrising.me/threads/crowhurst-paralysis-study-46-patient-responses.24818/

 

[Rufous McKinney]

Before onset, when I had smoked too much weed too fast

oh: that did happen to me once. About 53 years ago. Driving. Became paralyzed. How awkward. Fortunately I was in the boon docks. It never happened again.