Hi guys,

Please forgive me if I have posted to the wrong group but I'm pretty certain I am in the right place.

I am 44 years old and for the past 7 years I have been experiencing some pretty awful autonomic problems which seem to come in waves. I have a permanent tachycardia with a resting heart rate of 110 which at times can be bradycardic as low as 40bpm. I have orthostatic hypotension but the attacks are so transient as to not cause syncope although I can get dizzy if I repeat triggers such as straining or arm movements, I can extend the duration of blood pressure drops by maintaining an unstable posture which is the only way I could sustain them long enough to be measured. I have sporadic POTS. I have had a few instances of diplopia. I suffer from task based confusion and general fuzzy headedness. I have had inaccurate swallowing although not severe, I have sporadic numbness in the ulnar nerve bilaterally, and sporadic poor pupil response causing photosensitive eye pain and headaches sometimes having to wear sunglasses to use the computer or watch tv. I have inspiratory gasps particularly on transitioning to sleep when I also experience some very unpleasant cardiac symptoms that feel like my heart has stopped and some apnea and very loud snoring. I have the same feeling as normal people have on waking for a few seconds that in my case can last several hours or even all day. I have recurrent nail indentations like Beaus Lines although they are wide and undulating. I have reflux that has caused me to inhale stomach acids in my sleep. I have very disturbed sleep and occasionally wild dreams. I get a good deal of chest pain and headaches and occasional back pain. I have periods of rapid oscillation of heart rate and blood pressure. I have suffered a severe episode of syncope resulting in fitting and left twitching for hours afterwards when beta blocked down to 60bpm. I have very mild urinary incompletion and difficulty initiating, and no erectile disfunction. I have had some constipation and bloating and diarrhoea although mild and rare, and severe exercise intolerance

The condition is sporadic usually having one large attack annually lasting up to six months followed by several "aftershocks" interspersed with periods of partial "remission". Each attack is different resulting in patches of each symptom lasting about 2 weeks before fading and being replaced by a different combination of symptoms.

Initially I thought I might have MSA but progression seems to be slower, my age is unusual and I have no motor symptoms. I was also able to tolerate propofol with only some variable heart rate and chest pain upon waking which I assumed would be a problem if my condition involved CNS. My history includes exposure to an unusual flu brought back from Indonesia by a backpacking cousin which caused my first episode of syncope and was followed within weeks by my first episode of diplopia. This combined with pleuritic pain and some thickening of the skin on my feet led my to consider the possibility that this may be an autoimmune mediated autonomic neuropathy. Not only that but attacks have been accompanied by very hard lumps like chickpeas in my arm-pits and wrists and always with painful glands in my neck near the carotid sinus and occasional carotid sinus sensitivity. Sometimes attacks are initiated by mumps like symptoms and exposure to cold or flu viruses which are destroyed in hours. I have also noticed that a marked improvement of symptoms can be achieved by riding my motorbike which is like Lorenzo's Oil which I assume is the result of the generation of catecholomines.

I would be very interested in any feedback on this perplexing and extremely debilitating condition as it doesn't fit nicely into any particular autonomic condition having elements of many. I have had an abysmal experience of the medical profession to date having been dealt with initially as a psychiatric case and even accused of being a malingerer until I finally persuaded my GP to refer me to a cardiologist who confirmed a neurological condition that definitively ruled out a psychiatric etiology.


Many thanks

First let me welcome you, but also sorry you had to come here for help. You obviously have an extensive history of symptoms.

It does appear just from reading your post that you do indeed have some significant autonomic dysfunction. However, you don't mention any specific testing. What testing have you had done and what where the results?

I'm also curious what autoimmune testing you have had since you mention the flu as a trigger. Autoimmune conditions are often triggered by a virus or bacterial infection.

I understand the frustration with the medical profession...particularly with conditions that wax & wane. You really need to have some autonomic testing though. A tilt table would confirm the POTS and orthostatic hypotension. A 24-48 hour holter and/or event recorder could pin-point the heart rate issues and determine if IST (inappropriate sinus tachycardia) is present. A 24 hours ambulatory BP could also be helpful with a journal noting times of symptoms to see if problems correlate to drops in BP...and just how far it's dropping. A sweat test might be helpful but you don't mention and temperature regulation issues.

The double vision can be associated with dysautonomia, but you should have a thorough eye exam from an ophthalmologist just to be sure nothing else is going on.

Also, a gastric empty test might prove quite helpful to determine if your GI motility is impaired.

There are various urodynamic studies that can be done, but frankly I think the patient is usually competent enough to report difficulty initiating urination and retention without specific testing. Although a post-void ultrasound can determine exactly how profound the retention is.

None of the testing is invasive, but it is time consuming, expensive, and needs to be done by someone with proper experience.

Along with testing, you should investigate known possible causes of autonomic neuropathy/dysautonomia. One should be diabetes, especially with your complaint of double vision. Have you had a glucose tolerance test or A1C?

I would also consider some basic autoimmune blood work. Have you seen a rheumatologist?

It is not always possible to determine the cause. Then you just need to focus on treatment and relieving your symptoms. You really need to know more about your heart rate & BP before you start adding things like beta blockers, Midodrine or florinef. If your heart rate is increasing from a compensatory reaction to a drop in BP, then addressing the BP might help the heart rate without medication. Some people have both NMH (neurally mediated hypotention) or orthostatic hypotension & POTS or IST. Then you may need to look at treating both. There are also supplements that some have found helpful...like B12 & benfotiamine. I have tried them but they were not helpful...BUT my dysautonomia/autonomic neuropathy is from Sjogren's damage to the autonomic & dorsal root ganglia. Once the damage to the ganglia occurs, it is typically permanent, and symptomatic treatment is the only option. The BEST treatment is to address the underlying cause...in EARLY stages. So i would encourage you to seek out second opinions and testing (if you have not had any testing).

Sorry about the delay in replying, I was a bit too brain fried to get my head around this yesterday.

I have had some testing, the cardiologist confirmed IST. I have had ambulatory BP testing but if I was moving it registered errors because its too labile to measure and if I stood still my BP returned to normal. Consequently all they got was normal results and a load of errors. I have been tested for diabetes and tested negative.

I had a tilt table test when I first became symptomatic years ago which was negative as I was in a remission period and my condition was primarily cardiac and OH barely noticeable at the time. (I suspect this was because I get highly transient multiple blood pressure drops but only when moving. I have managed to have orthostatic hypotension measured since when I discovered I could prevent the normally rapid bounce back by maintaining an unstable posture and rocked back on my heels when being measured after standing.) Of course no positive tilt table result or ambulatory BP = no orthostatic hypotension. This resulted in an autonomic disorder being discounted (prior to the cardiologist result) and my GP promptly beta blocked me to deal with the tachycardia resulting in a catastrophic vaso-vagal syncope that caused fitting and left me twitching for hours afterwards.

Consequently I am mistrustful of overly reductive one-size-fits-all testing. I cannot be sure many of my symptoms are going to be detectable on a given day. Its safer for doctors to be uncertain of my condition than to perform a test, get me on a good day, discount my reported symptom and come close to killing me with dangerously inappropriate medication.

So I have spent the last few years researching all the various possibilities, MSA PAF AAG etc. and am very familiar with the testing regimes from clonidine tolerance to pupil response and even waded through Roger Bannisters textbook on Autonomic Failure. I have a medico-legal background so although my knowledge of medicine is rudimentary to say the least having mostly dealt with industrial accidents PTSD, orthopaedic injuries and mesothelioma I can at least digest the basics although I'll admit autonomic neurology is a stretch. I pursued the cardiological component of my condition because I purchased my own testing equipment referred to the book, knew it was almost always present, indisputable and easily confirmed, and so it proved to be. It took me 5 years from the detection of a resting heart rate of 110 to get IST confirmed by a cardiologist, by which time I had long since worked it out for myself. Symptoms like the sleep disorders are highly sporadic and happen for a few weeks 3 or 4 times a year, so I've no idea whether or not they will be present if I was to organise a sleep study and wait several months for the appointment.

What I am after here is answers to questions like does anyone else have the sort of highly transient orthostatic hypotension I experience, if so can I rely on a tilt table test to detect them? Do I need to be having an attack for AAG bloodwork to detect the problems? And what have people with conditions similar to mine been diagnosed with? My quality of life is considerably better relying on the many coping mechanisms I have developed myself and doing my best to enjoy life as best I can, than embark on another round of symptom exacerbating, adversarial medicine, and risk another near fatal cock up. So I am hoping to gather as much information as possible, narrow it down as much as possible so I know what testing I can undertake without making my situation worse.

Thanks again for your reply

Hello Osric,

Some of your symptoms sound very similar to mine. I recently went to Vanderbilt's dysautonomia clinic and was told that I do not have dysautonomia although I have tested positive on a paraneoplastic panel and fell in the range for dysautonomia. But, when I was at Vanderbilt, I was having an almost complete remission of symptoms and had been exercising heavliy for about 5 months. I had a positive hit on a tilt table test at one hospital months before, but not at Vanderbilt. I also did not have an issue with the other tests that were given, so the sweat test was eliminated from my visit. The doctor did say I my have mild orthostatic hypotension.
I have yet to pass out...

My resting BP seems to be fine, but I do get lightheaded when standing, but not all the time. Some blurred vision. I also have a panicked like feeling when falling asleep and sometimes gasp for air. I had a positive hit on a tilt table test at one hospital, but not at Vanderbilt. I also did not have an issue with the other tests that were given, so the sweat test was eliminated from my visit.
I also suffer from task based confusion, but not all the time.
I have pain basically all over my body from working out for 23 years and so every thing seems to hurt now. I also took Humira for about 10 years for psoriasis, this could have contributed to my neuropathy issues.
My pain is certainly affected by stress and unhappiness, two things I deal with almost everyday.

Let's keep in touch and continue to compare notes.

Nervous1

To simply answer your first question: YES, it is not only common, but expected, to have autonomic symptoms that come & go. Most of us with dysautonomia have waves of symptoms that last days/weeks or months. It's best to be tested during this time.

As for the OH and errors on readings while moving. It should still be accurate if you stop moving as soon as the cuff finishes inflating. If your problem is just when moving, then request a treadmill stress test. They take BP's and heart rates while you are walking...yet your arm is stationary/still on the bar in front of you so no errors will occur. You can also try BP's at home doing things you know makes it drop and just hold your arm still or be still once it inflates. Make a journal of activity that causes symptoms and record readings that correlate to it. Then repeat these in the doctors office.

I don't think AAG antibodies vary...they are either there or they are not...as in most other autoimmune diseases (whether in a flare or not). So I don't think your would have to be in an active state for them to show up. BTW, did you have a workup for autoimmune conditions?

I understand your frustration with one-size fits all for testing...particularly with a condition that symptoms wax & wane. But it appears they responded (not discounted) to your reported symptoms of IST and treated with what is usually a first line treatment...beta blocker. It's unfortunate that your had a negative reaction. Are you still having problems with the tachy rates? Is 110 the highest? Have they tried other medications?

Sorry I can't be of more help. Your BP is unusual and the fact that it returns to normal when standing still (vs drops) is sort of outside the box for OH. However labile BP can be problematic also. You will just have to practice various techniques to record BP's in different situations/activities. Most home BP monitors save the history for some time period. When you've collected numerous readings that show your problem, then take the machine with you and scan through the history to show the doctor the actual readings. Maybe this alone will help avoid further (hit or miss) testing. A good autonomic specialist KNOWS that symptoms come & go and should take this into account when testing and setting up testing during those time periods.

Thanks again for your help en bloc. The reason why the inflater cuff registers errors is because my blood pressure is changing during the measuring process. I suspect the errors are the result of the diastolic registering as being higher than systolic because the systolic is measured in a trough and diastolic at a peak. This has resulted in implausible readings like 160/60 and 110/100 where in the gap between measuring the diastolic and systolic my blood pressure is changing. It is extremely labile during the period of testing. My blood pressure drops last a second or two before bouncing back with an brief hypertensive overcorrection. I can sustain the blood pressure drops for an extended period if I initiate a trigger such as standing up and maintain the trigger with a sustained unstable posture. Of course the doctors assumed the errors were as a result of my arm moving or flexing and fouling the test. This is not the case, I am acutely aware of this issue and make sure I don't flex or wave my arms around during measuring and keep it steady during inflation.

I do however, during attacks, experience a more orthodox OH where my baseline blood-pressure drops, and I experience dizzy spells, accompanied by comparatively mild POTS. I'm pretty certain my OH, whilst as you say is a little outside the box, is simply a result of my being fortunate that I require a little more orthostatic stress than usual to trigger a drop. So for instance I just measured my BP and it was 140/80 seated 145/90 after standing( probably a bounce back overcorrection) and 105/70 after walking up the stairs. I had a 15bpm increase on standing despite such a highly transient bp drop. Similarly I can stand at the washing up sink fine, but when I start moving my arms around to perform the task my blood pressure starts bouncing around. I should of course be grateful for the improved functionality of this but it certainly has made diagnosing it a misery.

The beta blocking incident occurred before the IST diagnosis as a result of the assumption that my tachycardia was a psychiatric issue having discounted the blood pressure issues. I was beta blocked to 60 bpm with the sort of beta blockers used for psychiatric problems. Following the IST detection I was beta blocked using a very small dose of bisoprolol and only down to 90bpm, which I was able to tolerate and provided some relief, although it works against me when I'm bradycardic. I have never had panic attack like symptoms. Just a highly stable 110bpm the highest resting heart rate was around 130bpm with rare very unpleasant chaotic attacks with wildly fluctuating BP and heart rate. This is an extremely hair raising experience and at times I have had to lie on the floor and elevate the legs to maintain consciousness and I just take 300mg of aspirin and hope for the best. I'm not sure I share your opinion of my medical care to date. There is no excuse for detecting a resting heart rate of 110 and it taking 5 years to get a referral to a cardiologist. A patient shouldn't have to purchase his own cardiac monitoring equipment, study autonomic neurology, and correctly diagnose himself to convince a doctor that a resting heart rate of 110 warrants a referral to a cardiologist. Since the IST was detected I have a better relationship with the medical profession but I am still wary of undergoing testing that could throw up misleading results. Once bitten twice shy, and I think you can see how my blood pressure issues can circumvent standard testing.

I don't think my gasping is quite the same as yours Nervous1, although I know exactly what you mean. I am aware of the nature of the inspiratory gasps because they occur whilst I am still awake. They are not me waking up with a start. I am breathing normally and I will suddenly take a sharp involuntary intake of breath. This is a very common symptom with MSA as a result of the brain failing to detect breathing - the reverse of central sleep apnea. I have also had episodes whilst asleep where I wake and take an absolutely vast lungful of air similar to if you exhale far beyond your normal cut-off which I suspect is a failure to shut off the exhale reflex. These are concerning symptoms as as far as I am aware they are closely associated with CNS autonomic dysfunction. I would be very pleased to here from anyone experiencing this particular symptom with a non CNS diagnosis. Perhaps I should take this particular issue elsewhere.

Thankyou so much Nervous1 for sharing you personal experiences. It really helps to hear others experience and cross-reference with my own. Its interesting to note autonomic disruption not associated with dysautomnia, there were autonomic components of PTSD cases I've come across.

I very much appreciate your input on the AAG testing en bloc. I think as a result I will go ahead with them. It is precisely the sort of input I was hoping for. It is a great comfort to have a place to go with my queries. I'm sorry if I come across as somewhat prickly at times, its been a very frightening, isolating and frustrating few years and just talking about it takes me to a very painful place.

I'm a bit more clear now on your BP. I'd still suggest the stress test as I think your heart function needs more investigation and the BP and heart rate can be closely monitored...and a doctor is always present during the entire test and therefore could see first hand what is happening and not be able to dispute the findings.

Trust me, I have no illusions about how frustrating and unpleasant medical care can be. It took me 12 years to be properly diagnosed with neurologic complications of Sjogren's. I had a collection of various autonomic dysfunctions, PN, weakness, joint pain, and numerous other symptoms and separate diagnosis, including strokes, open heart surgery...the list goes on. I was bounced around from doctor to doctor, including the Mayo Clinic. No one could figure out the one component that was causing everything...because my labs for autoimmune disease are sero-negative (now I know that 40% of patients fit in this category). It wasn't until I went to Johns Hopkins for a consult about my antiphospholipid syndrome (APS) and 3 strokes that a doctor sat with me for 3 hours reviewing my extensive history. He ended saying, I think you have Sjogren's and that it's attacked your nervous system. A lip biopsy confirmed his thought, and a special MRI/MRN confirmed the damage to my dorsal root ganglia. 12 years it took for that first test to put all the pieces together! I assumed you meant the beta blocking experience was after your IST diagnosis...which would have been an appropriate treatment. I'm on the same page now.

I'm curious about your OH. What happens after you stand for 2-3 minutes? After 5-10 minutes? How low does your BP go? If your BP goes back to normal after standing, then how long do these attacks last? Is the worst of the attack during the labile BP or low BP? Do you experience any chest pain/discomfort during these attacks? If so, describe it, please. I ask because I wonder if you're experienceing any cardiac vasospasms? This is yet another autonomic dysfunction that can occur and can greatly affect your BP (both high & low) and heart rate. Also, do you have any unusual skin discoloration (particularly when cold)...a lacy pattern of blood vessels. It's called livedo reticularis. If you google images, you'll see what it looks like. It also a sign of blood vessel spasms (in medium blood vessels of the skin)..and of course an autonomic dysfunction. They are not widely spoken of, but most definitely an autonomic dysfunction. The cardiac vasospasms are difficult to diagnose, but an echo stress test may be helpful if they see any heart wall dysfunction, absent of severe CAD.

BTW, I have experienced the gasping upon onset of sleep. I too am still actually awake and realize it happening. My pulmonologist says it falls in the category of central sleep apnea and directly related to the dysautonomia. He said, in central apnea, the brain forgets to breathe. Have you had a sleep study done? Mine thankfully is a rare occurrence, so would likely be impossible to document on any given night. When mine occurs, it is also in waves for a couple days or even several times on the same night...sometimes waking me up with the gasping.

Is there anything you can think of when this began that might help lead you to the cause...it sure would make diagnosis much easier if you knew what brought it on. Was the onset sudden or gradual? Did it occur right after the flu bug you mentioned? What about taking any antibiotics or around any unusual toxins? If this began suddenly and after the infection, I would certainly consider autoimmune. Have you had any AI testing? Did you see an infectious disease specialist about the flu bug?