Temporary paralysis I need your Insights

[Nemesis]

@Nemesis I do get migraines, although a lot less on POTS meds. How long have you had temporary paralysis at a time? Is it with other migraine symptoms? I'm generally intolerant of electric light but that day I was ok with hospital lights.

Strange thing that after my pots my hemeglebic migraine completely gone just migraine without paralyzing, my migraine decreased don't know how or why, well generally white light is trigger my migraine more than yellow lights but if yellow light glare it will trigger migraine , well for more than 6 years didn't have any paralyzing problems but as far as I remember it last for 4 to 8 or 6 hours

 

[Jenny TipsforME]

@Nemesis there seems to be some POTS association with this experience I think. Both the ER and my POTS doctor were adamant it's not to do with POTS. I agree that it isn't typical POTS itself but I wonder if there's some reason POTS makes it more likely (e.g. sodium/potassium channelopathy or acetylcholine receptor issues or POTS caused migraine)? To me bad ME and bad POTS feel qualitatively different (though they can aggravate each other) and it felt like POTS. If my ME had been severe I wouldn't have been able to explain properly as I'd be really foggy and pausey and I would have wanted darkness. Also ME flares can come on suddenly but I've not known ME weakness stop suddenly.

 

[Nemesis]

@Nemesis there seems to be some POTS association with this experience I think. Both the ER and my POTS doctor were adamant it's not to do with POTS. I agree that it isn't typical POTS itself but I wonder if there's some reason POTS makes it more likely (e.g. sodium/potassium channelopathy or acetylcholine receptor issues or POTS caused migraine)? To me bad ME and bad POTS feel qualitatively different (though they can aggravate each other) and it felt like POTS. If my ME had been severe I wouldn't have been able to explain properly as I'd be really foggy and pausey and I would have wanted darkness. Also ME flares can come on suddenly but I've not known ME weakness stop suddenly.

My hemeglebic migraine was sever and cause me paralyzing and brain fog make me like drunk, it's gone suddenly but gradually after that my pots appeared

 

[Jenny TipsforME]

@Nemesis oh so you got POTS after this problem resolved?

 

[Nemesis]

@Nemesis oh so you got POTS after this problem resolved?

Yes

 

[kangaSue]

I agree that it isn't typical POTS itself but I wonder if there's some reason POTS makes it more likely (e.g. sodium/potassium channelopathy or acetylcholine receptor issues or POTS caused migraine)?

Unpublished data from a Mayo study found that 25% of those with POTS had alpha3 nicotonic acetylcholine receptor antibodies. Voltage gated calcium channel and potassium channel can be pathogenic and are being more looked at with autonomic issues now and I would suggest that anyone with autonomic dysfunction get tested for all three of these antibodies at the least. Raised ANA and or GAD65 or often present too.

 

[Jenny TipsforME]

@kangaSue thanks.

I've got my first neurology appointment in a couple of weeks. I usually prepare for appointments and take notes in with me (chance of brain fog wasting the appointment otherwise high). The AchR tests are something I plan to ask about. I'm going to go back through this thread and jot things down.

Does anyone else have ideas on what I should ask (especially if you have a sense of what is realistic with the NHS)?

I have printouts of my SNPs possibly related to periodic paralysis. Is this going to be perceived as neurotic or helpful if I take it with me?!

 

[Gingergrrl]

Unpublished data from a Mayo study found that 25% of those with POTS had alpha3 nicotonic acetylcholine receptor antibodies. Voltage gated calcium channel and potassium channel can be pathogenic and are being more looked at with autonomic issues now and I would suggest that anyone with autonomic dysfunction get tested for all three of these antibodies at the least. Raised ANA and or GAD65 or often present too.

Very interesting @kangaSue and I have POTS but have the autoantibodies to the muscarinic receptors (not the nicotinic ones). I also have the autoantibodies to the N-type CA+ channels but not to the Potassium channels. And for good measure, I have a positive ANA (1:160 speckled type after at least five years of the ANA being negative) and I am positive for GAD65. I have never had any episodes of paralysis but have POTS/autonomic dysfunction, breathing weakness, and muscle weakness- all of which are improving with high dose IVIG.

@Jenny TipsforME I am hoping that you can get the testing that you need and it sounds like the Potassium antibody tests are crucial with the episodes of paralysis that you experienced. I have no idea what you can get on the NHS (being in the US) but from everything I've ever read on PR, I'd guess the odds are low and you'd need to see a private doctor and hope that they can send the blood tests to Angela Vincent's lab (in the UK) or to Mayo Clinic (in the US). Please keep us posted.

ETA: I don't know the answer to your question re: SNP's but very few doctors that I have seen know much about 23andMe or SNP's and my gut instinct is to focus on getting the testing regardless of the SNP's. In my own case, I do not know if my SNP's would add anything useful b/c my problems are not life-long and all began about four yrs ago.

 

[kangaSue]

Very interesting @kangaSue and I have POTS but have the autoantibodies to the muscarinic receptors (not the nicotinic ones). I also have the autoantibodies to the N-type CA+ channels but not to the Potassium channels. And for good measure, I have a positive ANA (1:160 speckled type after at least five years of the ANA being negative) and I am positive for GAD65. I have never had any episodes of paralysis but have POTS/autonomic dysfunction, breathing weakness, and muscle weakness- all of which are improving with high dose IVIG.

@Gingergrrl It would be being greedy if you had the full complement wouldn't it?

I would think that paralysis events are more likely to occur with high titres of antibodies. N-type voltage gated calcium channel Ab also regularly shows up in studies I've seen into antibodies occurring in people with POTS. Rituximab is looking to be a good option for you to try with your collection of antibodies.

 

[anciendaze]

One problem with medical thinking about "diagnostic tripwires" is that doctors seem to assume problems must be confined to single measurements, which are interpreted linearly. This ought to seem absurd on the face of it, but it is especially absurd when you understand that many of these antibodies interact with the same cellular features or components.

The neuromuscular junctions likely behind many cases of unexplained paralysis are tiny features connecting nerve cells with muscle fibers across a synapse measured in nanometers, (like high-tech electronics.) There are four clear-cut ways these can fail: the calcium channel required for vesicles holding acetylcholine to fuse with the presynaptic membrane and release contents may be blocked; the receptor on the postsynaptic side may be blocked; the muscle-specific protein kinase released when a molecule docks with the postsynaptic receptor may be depleted; the neurotransmitter which bounces back across the synaptic gap may not be picked up for reuptake, meaning it will not be available for later release.

Gingergrrl has what are considered minor problems with two of these, but no one seems to think these interact. Chemical transmission of nerve impulses at neuromuscular junctions was discovered in the 1930s. Acetylcholine was the first known neurotransmitter. If the medical profession still doesn't have a model for how impairment of different parts of the process combines to produce a clinical effect, and isn't bothered by this ignorance, something is wrong with the profession.

Patients who experience real temporary paralysis because of impairment of receptors for potassium, sodium and/or calcium are in an even more difficult situation to analyze. I believe the incidence of such problems has been systematically underestimated.

 

[Jenny TipsforME]

I routinely have problems walking/standing which I attribute to POTS. But my cardiologist isn't keen to accept "concrete legs" as part of POTS. He does accept that he has other patients with POTS who seem to experience the same problem. I'm not quite sure if this is semantics!

I can walk a few metres with normal strength. E.g. I can walk up our steep stairs without hesitation most days. Fairly randomly, but enough that I can't leave the house without a wheelchair, my legs will suddenly go very heavy so it is impossible to walk. If I don't sit or lie down my legs will give way and force me down. It is less dramatic than not being able to move for 11hours but as it is routine, it is more disabling for me.

The impression I have is my cardiologist thinks this could be a milder version of the paralysis incident (neurology related), I also think they're related but because I think it is connected to POTS...

Reasons I've thought concrete legs POTS blood pooling related:

• I have other POTS symptoms at the same time (lightheaded etc), it seems to be a presyncope symptom warning me I'll faint if I ignore it
• Improves with POTS meds eg ivabradine
• Seems reliably triggered by vasodilation e.g. Hot bath, alcohol
• Seems to be the standing more than the moving which is problem e.g. Can move legs straight away when lie down (v different to the thing in May, then I couldn't even shuffle in bed for hours)
• Seems to respond to electrolytes. If I'm having a bad day drinking an electrolyte drink often improves my ability to stand

I might be wrong about this though. It could be a channelopathy sodium/potassium/calcium issue more than vasodilation (I know the signals are interlinked). Ivabradine effects the Funny channel (If)

If is a mixed Na+–K+ inward current activated by hyperpolarization and modulated by the autonomic nervous system.

wikipedia
I think it also involves acetylcholine and muscarinic receptors, but my brain isn't up to understanding that today.

All the things to do with electrolytes could point either way. What do you think?

I'm realising I have a bit of baggage because my successful diagnoses have been when I've worked out beforehand what is wrong and doctors have confirmed it. I don't really have experience of doctors reaching a diagnosis from scratch but I guess that is more normal!

 

[anciendaze]

@Jenny TipsforME

You are describing multiple problems linked to autonomic dysfunction, and you are dealing with a medical professional who concentrates on heart problems. This is not primarily a heart problem, but there is plenty of evidence of disturbed hemodynamics under conditions that don't show up in the clinic. (How many patients take hot baths with a doctor watching?)

Reduced blood flow exacerbates many problems with fluids, electrolytes, oxygen and glucose. I suspect that some of your symptoms would go away if your primary problem was corrected, but at this point I don't know how to do that.

I've heard doctors tell me what they think they know about POTS, and it is discouraging, because they are usually badly misinformed. Many concentrate on one aspect of POTS, tachycardia, and treat that as if it were in isolation. (There are patients like myself who do not experience tachycardia, but do show neurally-mediated hypotension over a period of minutes while standing. Even that term is misunderstood because the problem is not usually low systolic blood pressure, but low pulse pressure, systolic - diastolic.)

The primary concern of cardiologists dealing with autonomic problems is distinguishing events like vasovagal syncope from a true heart attack. Once they know that it will not lead to a death which can be blamed on them they lose the sharp focus of attention. Some will fall back on traditional explanations of psychosomatic illness for things they don't understand and can't control. (Many cardiologists could be described as control freaks.)

Even those who believe the patient has a real physiological disease do not have much training in dealing with chronic disease. The strong emphasis in that specialization is on avoiding or delaying mortality, and many patients with true heart disease end up in a morbid condition where they aren't good for much except lying in bed, possibly waiting for a heart transplant. You are in better shape than that, so your current condition is considered acceptable.

Asking a doctor with advanced training in such a specialization, and years of experience with patients who did end up as I've described, to reassess his/her career contribution to patient welfare is too much. If you considered those outcomes as failures the mental burden would be crushing.

 

[Jenny TipsforME]

@anciendaze in my area POTS is assigned to the cardiology team. When I go for appointments it's a POTS/syncope clinic in the cardiology department. I accept that the issues I raised at the last appointment aren't in the specialism of cardiology. Unfortunately our bodies don't respect these discipline boundaries!

I think because of their background knowledge there's an over emphasis on the tachycardia aspect as you say, often forgetting the "syndrome" components. It is also reassuringly measurable: he can see my heart rate and adjust it with drugs (in my 1st appointment with him it was 120 sitting, 150bpm standing, now it's 50s/60s at rest, 80-110bpm standing). I perceive that he does take my condition seriously. He seems to recognise that it's disabling, he just has a narrower definition of what POTS is than me. I see the tachycardia as a sign of the underlying POTS rather than the problem itself.

I wonder if neurologists running POTS clinics over emphasise the non cardiovascular bits?! The neurologist I'm going to see in a couple of weeks may not even know about POTS. He's an MS specialist. I don't hold out high hopes but he's mainly a researcher so may be a naturally curious type.

 

[anciendaze]

@Jenny TipsforME

Because antibodies to GAD65 shift the balance between glutamates and GABA, thus between sympathetic and parasympathetic activation, I've been looking into connections between a positive test for antibodies to GAD65 and dysautonomia. These antibodies also show a strong association with type 1 diabetes.

I just had the chance to talk to a type 1 diabetic I know who is sufficiently fragile so that doctors want to install an insulin pump. Does she have antibodies to GAD65? Never tested for this. Does she experience dysautonomia? Not by name. Does she have problems when she stands up suddenly? Yes. She has been told to manage fluids and electrolytes carefully to avoid syncope when standing. She understood orthostatic intolerance immediately when I described my problem.

There are also definite parallels in periodic paralysis (PP) patients, though those with both PP and type 1 diabetes are at high risk of dying, and thus rare.

Antibodies to GAD also turn up in stiff-person syndrome, which may or may not be a form of PP.

I will also report that I have yet to find a patient with a positive test for GAD65 and no other positive test for autoantibodies, when these have been run. This is part of a general autoimmune disorder.

For most medical professionals these are entirely distinct and unrelated disorders. None of these are primarily heart problems, but several patients I'm aware of are being treated by cardiologists.

 

[Gingergrrl]

Am just catching up on Jenny's thread and apologize for the delayed reply!

@Gingergrrl It would be being greedy if you had the full complement wouldn't it?

@kangaSue Thanks for making me laugh! ITA that it would be greedy if I had every single autoantibody on these tests even though lately it feels as if I do LOL!

N-type voltage gated calcium channel Ab also regularly shows up in studies I've seen into antibodies occurring in people with POTS. Rituximab is looking to be a good option for you to try with your collection of antibodies.

That is so interesting re: the N-type voltage gated calcium Abs regularly showing up in studies w/people who have POTS. I wish actual doctors knew about this stuff. I agree with you that RTX is looking like a very good option for me next year after I finish my six months of high dose IVIG. The IVIG has already brought me improvements that have exceeded my expectations and I plan to post a new thread on this after my next round of IVIG which is Jan 5th, 6th & 7th. I want to share everything I have learned and will have more time to post at that point.

Gingergrrl has what are considered minor problems with two of these, but no one seems to think these interact.

@anciendaze, you could not be more right that no one except for my ME/CFS doctor understands that there is a connection between my rare autoantibodies and my symptoms. He was certain that high dose IVIG could help me and he was absolutely right.

Patients who experience real temporary paralysis because of impairment of receptors for potassium, sodium and/or calcium are in an even more difficult situation to analyze. I believe the incidence of such problems has been systematically underestimated.

I agree that this is even more rare and I wish people could get tested for these channelopathies to find out what the real numbers are and then compare them with each person's symptoms. We are on to something here for sure but doctors do not take this seriously (assuming they have even heard of this issue). Am not kidding that I know more about this than the average doctor and we all know how minimal my science background is (or was).

@anciendaze in my area POTS is assigned to the cardiology team.

Jenny, POTS is almost always assigned to cardiology here as well but most cardios know next to nothing about it. The first cardio I saw in 2013 thought it was absolutely fine that my HR was in the 160's and 170's on a daily basis. She said to me that "Her HR often goes that high during her exercise class" and I said yes but mine is going that high while I am sleeping or b/c I stood up from a chair!!! Let's just say that I quickly moved on to cardio #2 (and am now on #3).

I think because of their background knowledge there's an over emphasis on the tachycardia aspect as you say, often forgetting the "syndrome" components.

The only thing the cardios have ever been interested in is the tachycardia (which is controlled with a beta blocker for me) and occasionally on my hypotension which was in the 80's/50's on a daily basis before I started IVIG.

I wonder if neurologists running POTS clinics over emphasise the non cardiovascular bits?! The neurologist I'm going to see in a couple of weeks may not even know about POTS.

The Neuro that I saw at beginning of 2016 was the worst medical experience of my entire life. He found I had significant POTS on TTT in addition to finding that I had the two autoantibodies (N-type CA+ channel and anti GAD65) plus found a weird abnormality on an EMG. But b/c I have a mast cell disease (which he did not believe existed) he decided my entire case was "psychosomatic" and refused to treat me and sent nasty letters to my main doc and MCAS doc. Luckily both of those docs know me well (and know how ill I was that I've required a wheelchair for 2+ years and how much I was suffering) and disregarded the letters as complete nonsense. I was able to start (low dose) IVIG back in July and increase to moderate dose in Nov and high dose in Dec. I am hoping that your experience with the Neuro is better than mine (although can't imagine that it could be worse)!

Because antibodies to GAD65 shift the balance between glutamates and GABA, thus between sympathetic and parasympathetic activation, I've been looking into connections between a positive test for antibodies to GAD65 and dysautonomia.

I would love to learn more about the connection between GAD65 and dysautonomia b/c I feel it is exactly what was occurring in my case (since I do not have diabetes). We wondered briefly if I had Stiff Person Syndrome (SPS) but I am now certain that I do not. So dysautonomia now seems like the most likely explanation.

I will also report that I have yet to find a patient with a positive test for GAD65 and no other positive test for autoantibodies, when these have been run. This is part of a general autoimmune disorder.

That is also very interesting since I have eleven autoantibodies total with GAD65 being only one of them. I belong to two private groups (one for people with the CA+ antibody) and most people do have MANY different antibodies although only a few of us have the GAD65. It seems that the people who only have one autoantibody are more likely to have a paraneoplastic syndrome/cancer vs. those of us with multiple auto Abs, do not have a PNS vs. more general autoimmune chaos that (so far) has no distinct name. I am certain that some day what I have will have a name even if it does not at this point in time.

 

[Jenny TipsforME]

I'm feeling nervous about finally having a neurology appointment tomorrow.

I've made notes and decided what to wear. Do other people think through what they wear to doctor appointments or is that a personal weirdness? I'm aiming to look like someone he can relate to, he's a doctor so I suspect the women in his life are middle class. I don't want to make too much effort though because I am ill and don't generally have the energy to (so wearing makeup may give the impression of being healthier than I am).

Anyway I've no idea how it will go

 

[Jenny TipsforME]

Well I had the appointment and it was a nice neurologist who listened well. He's testing me for myasthenia gravis ab, some general blood tests and an EMG as a first step to working out what is going on. He did seem convinced that something is going on but he wasn't entirely sure that it will be in the sphere of current medical knowledge (which is a healthier attitude than "I don't know the answer so therefore this is psychosomatic ").

My legs are bad today which was actually quite useful for demonstration purposes! I had to shuffle from wheelchair to the examining couch. Some of the neuro exam tests I think I was fine on and others I couldn't do at all. I couldn't do the lying down raising legs straight one at all (IIRC there's a whole thread on here about that as lots of us can't do it). There's one I don't remember doing before: put your foot on opposite knee and move down towards that foot. I couldn't do it at all. I was slightly off with the eyes closed finger to your nose test.

Intriguingly the doctor didn't know what this pattern meant. He did seem convinced it meant something though!

Any ideas about this neuro exam? e.g. @anciendaze @Valentijn @Gingergrrl @kangaSue

 

[Jenny TipsforME]

This was one I couldn't do at all. Can you do it?!

"Co-ordination
Heel to shin test – “run your heel down the other leg from the knee and repeat in a smooth motion”

An inability to perform this test may suggest loss of motor strength, proprioception or a cerebellar disorder."

So this is a co-ordination/proprioception test too, like the finger to nose with eyes closed one, but I think you're just meant to fail by not keeping your heel on the other leg, whereas I couldn't move my leg at all in this way.

 

[anciendaze]

@Jenny TipsforME

Yes, I can perform that test. My orthostatic intolerance is not primarily due to my legs. My back aches, but the real limitation shows up when my brain gets shortchanged of oxygenated blood.

In my non-expert opinion you show real evidence of a neurological problem, though there are still questions about neuromuscular junctions and metabolism to answer. At this point I'm having trouble localizing this to a particular part of the nervous system. It points to real problems somewhere in your spine (spinal stenosis?) or the lower parts of your brain. There are quite a number of ways things can go wrong, simply from a mechanical standpoint, with soft material that does not show well on X-rays impinging on nerves. At this point it is not even easy to say "run an MRI on every vertebra" for diagnosis, because the evidence may depend on upright posture. There are also possible problems with blood flow or cerebrospinal fluid, even before we get into questions about metabolism, inflammation or infection. What a good doctor will look for are problems open to interventions.

Here's hoping you have reached such a doctor who will find a problem that can be treated.

 

[Jenny TipsforME]

@anciendaze the doctor I saw today is mainly an academic researcher so I'm hoping that could mean he has the curiosity and tenacity to follow the trail to some sort of destination. I was a bit worried that he mentioned the limits of medical science though. I realise this is very true, but an expectation of whether the answer is findable will probably influence the nature of the search for it.

For the leg/walking/standing issue I've put that down to POTS for the last couple of years (it usually comes with feeling lightheaded) but the two lying down tests I couldn't do today wouldn't be that, as not standing/sitting. The leg issues fluctuate quite a bit so I'll probably try those tests again on a good day.