What Is POTS??

What Is POTS??

Monday, October 3, 2016

CAN A CHIROPRACTOR TREAT POTS?

I recently watched a video that was posted in a POTS support group. The man on the video is a chiropractor, Dr. John Bergman.

The makes it sound like things like beta blockers and tricyclic antidepressants are how POTS is typically treated. Later in the video he seems to imply that he can cure or treat POTS by doing chiropractic manipulation.



He is a teacher at a chiropractic college, but he doesn't specialize in either cardiology or neurology or even internal medicine.
http://bergmanchiropractic.com/meet-the-chiropractor.html

He does teach physiology, an offshoot of biology. But he really doesn't go into how the organs work much. His other field is biomechanics which is more akin to chiropractics than anything to do with neurology and internal organs. He also lists anatomy, but he pretty much skips over how the organs affect POTS.

I don't buy into the idea that you can use chiropractics for the treatment of orthostatic intolerance. And I hope you will consider all of the information here that disagrees with it. There are too many other mechanisms involved and you need to know what is causing YOUR POTS as opposed to someone else's. After you read this post, if you still think chiropractics is the way to go, there isn't much else I can say to disuade you.
Underlying Causes of POTS
What Is Causing Your POTS? And Why It IS A BIG DEAL: A Printable, Sourced Guide

Dr. Bergman gives very rudimentary explanations for the mechanisms that cause POTS. They are not always the same for every patient. That is why it is called a Syndrome vs Disease.

Not all POTS patients have low blood volume. It is very important to identify the type of POTS or the underlying condition that may be causing POTS because it can help  your doctor decide what strategy to take for treatment. Different types of POTS include neuropathic POTS, hypovolemic POTS, hyperadrenergic POTS and POTS to due to de-conditioning. Some people have
more than one of these that cause their POTS. People with neuropathic POTS can have adrenal impairment and sudomotor denervation, which is nerve damage. This is why treatment is never a
one size fits all thing. There are even people who have autoimmune causes for their POTS, like
autoimmune autonomic ganglionopathy
POTS Subtype: Does It Really Matter?
"Another problem with these labels is that they are not mutually exclusive."

One of the first things he says is that doctors don't know what causes reduced blood flow returning to the heart. He quotes a NINDS Postural Tachycardia Syndrome Information Page But the dysautonomia information network gives an explanation for it. POTS mechanisms: dysautonomia information network It could be that no matter how fast the heart beats if the blood vessels in the lower body don't constrict enough to raise blood pressure, not enough

Beta blockers-- also known as beta-adrenergic blocking agents are drugs that block norepinephrine and epinephrine (adrenaline) from binding to beta receptors on nerves. Now just from that definition, you can understand that something is wrong with the adrenaline system or the receptors. He is indicating that they are bad for all patients and that they are routinely prescribed for all POTS patients, which they aren't. He says he watched a video or a speech where a Mayo Clinic doctor was saying that beta blockers work for most patients and that they work much better than calcium channel blockers. It is true that they work better than calcium channel blockers. But not that all doctors give them to all patients. And it isn't recommended to give ACE inhibitors or diuretics, but he has a slide up and makes it sound as if that is how it is commonly treated.  At least some of the doctors at the Mayo Clinic say to try nonpharmacologic treatments before beta blockers. Postural orthostatic tachycardia syndrome and chronic fatigue in adolescents: Working toward recovery

      "Beta blockers, including propranolol, block the receptors that are responsible
        for the effects of epinephrine and norepinephrine (catecholamines produced
        by the sympathetic system).We have shown in a placebo-controlled trial that
        propranolol decreases heart rate and acutely improves symptoms in patients
        with POTS. Interestingly, we found that symptoms were more improved by
        low dose rather than high dose propranolol."
Vanderbilt Autonomic Dysfunction Center--Propranolol

"The use of beta blockers in POTS is controversial, but we have seen many patients, including those who have previously failed  beta blocker therapy, have success with propranolol.
They shouldn't be prescribed for all patients. However, some do benefit from a low dose."

"Some patients, particularly, those with partial dysautonomic POTS, improve on small doses
 of beta blockers (e.g. metoprolol 25–50 mg once or twice daily), but the majority feel
worse on beta blockers." "The combined alpha1/beta blocker labetalol is useful in some
patients, as beta-blockade alone may worsen symptoms due to unopposed alpha-receptor
stimulation. A starting dose of 100–200 mg twice daily is used, and the maximum dose is
400 mg twice daily."
Postural Orthostatic Tachycardia Syndrome (POTS): A Diagnostic Dilemma

Well, logic would tell you that for some people they will work and for some they will make things worse. That's why it's good to find out what is causing the POTS. For those who do take them, the goal is to find a dosage that slows down the tachycardia without lowering the BP too far. Sometimes it is a very low dosage. He says that it is recommended that beta-blockers be used to treat POTS and that isn't true. It is only used for some patients, not all. The above link basically talks about how it is necessary to find out what the cause of the POTS is in order to treat it properly.

Dr. Bergman points out that the heart rate speeding up is a good thing because it is your body's way of making sure blood gets to your brain and says it doesn't make sense to use beta blockers because they will slow your heart rate down and then you will get less blood flow to the brain. But he leaves out a very important fact: Studies show that long-standing persistent tachycardia in certain conditions has a potential to induce a dilated cardiomyopathy (or an enlarged and weakened heart). Tachycardia-induced cardiomyopathy can result from prolonged periods of rapid heart rates. Granted these studies were on people that had tachycardia from things like Afib or ventricular tachycardia, but it is believed that the same thing can happen from the tachycardia in POTS. Basically,where he was wrong was in indicating that the heart rate just raised and not that it raises too high, which is why it sometimes requires treatment. A resting heart rate that is over 100 bpm is tachycardia.High Heart Rate Increases Risk Of Death, Even In Fit People  "resting heart rates over 90 beats per minute tripled the risk....
Every 10 to 22 additional beats per minute in resting heart rate raised the likelihood of death by 16%, overall, according to the authors." Myth: A Normal Heart Rate Is 60-100 beats per minute.
 Many doctors think it should be lower. About 50-70 beats per minute is ideal, says Suzanne Steinbaum, MD, director of women's heart health at Lenox Hill Hospital." 5 Heart Rate Myths Debunked But aside from that, you won't necessarily have low blood because of a lower heart rate. Busting 5 Myths About Blood Pressure and Heart Rate He says that anything that reduces oxygen to the brain, i.e. a lower heart rate is bad. He uses the example of a giraffe and says that the giraffe would have to have a higher heart rate to get oxygen to its brain. But having a high heart rate isn't good either. "Circulatory shock, commonly known as shock, is a life-threatening medical condition of low blood perfusion to tissues resulting in cellular injury and inadequate tissue function. The typical signs of shock are low blood pressure, rapid heart rate, signs of poor end-organ perfusion (i.e.: low urine output, confusion, or loss of consciousness), and weak pulses." Shock (circulatory) And then he says that water and appropriate nerve supply are the way to treat POTS. But those things can't always be fixed so easily either.

But it also necessary to understand how the autonomic system works and that it is not entirely controlled by the spine. You can't just fix the spine and think that will fix the whole system.

For instance, if you are one of the people who have serum auto-antibodies to alpha-3-acetylcholine receptors of the peripheral ganglia then you can't alter the autoimmune response by spinal manipulation.

It causes increased noradrenaline due to impaired clearance or decreased uptake of noradrenaline by the synaptic cleft. The synaptic cleft is a gap between neurons somewhere in your body. Not in your spine. Here's a video of how that works.
(Given how dumb that guy thinks people are, it's kind of amusing that the video
called A level biology. This is just basic biology/anatomy.)


The Brain—Lesson 2—How Neurotransmission Works



Hyperadrenergic state means that more adrenaline than usual is released, and then on top of that, it isn't reabsorbed like it should be. Again, you have both a gland that is being overactive as well as nerve receptors not working right either, i.e. some sort of neuropathy.

Selective serotonin reuptake inhibitors. The reason for those is because the nerves are not working properly and they basically reabsorb the serotonin before they should.You need the serotonin to help with vasoconstriction. And contrary to what he implies, it doesn't all originate in the gut. And some people are born with a genetic difference that causes their serotonin problem.

SSRIs will make existing bipolar worse, and that sometimes means that someone who was misdiagnosed with depression will suddenly become much more manic. But it doesn't cause them to be bipolar. That is an outdated idea. DSM-5 now says that it isn't the medicine it is that they were
misdiagnosed because bipolar often presents with several bouts of depression.

He points out that tricyclic antidepressants are on the list of drugs that make POTS worse, but those are an entirely different kind of antidepressant and work differently and he says they are one of the most prescribed drugs. But they really aren't recommended. POTS: What to Avoid He doesn't make clear whether or not he means in general or for POTS. There is no doubt that a certain percentage of people can become suicidal on antidepressants, but it's irresponsible to indicate that everyone or even a large percentage of people will or that they will become bipolar. For one reason, it's difficult to tell if a person who is put on an antidepressant might not have committed suicide regardless of whether or not they were put on medication. There is an increased risk in some studies, but in others there is a decreased risk. Antidepressants and Suicide in Adolescents and Adults

People usually become bipolar before the age of 25. He also says that the tricyclics are one of the most prescribed drugs for POTS. But that isn't true. SSRI and SNRI are the usual ones, particularly Celexa Autonomic Disorders: Syncope Dysautonomia & POTs or Postural Tachycardia Syndrome . That's why that list he was reading off said they would make POTS worse. It was so that doctors would be careful about using tricyclic antidepressants for POTS, not to suggest them for treatment. The dysautonomia information network says to avoid them. And they are quoting Dr. Blair Grubb at the University of Toledo Medical Center. He is one of the leading experts on POTS. So the experts are not recommending them as is implied.

I don't know if he figures the average person doesn't have a very good understanding of the body, or if he himself doesn't. I mean the way he presents the video is that he is trying to make a complicated subject easier to understand. But you don't do that by leaving out part of the information or being inaccurate.

He keeps saying that people just need more water and not beta blockers. Again, not everyone is prescribed beta blockers and some people have POTS because no matter how much water they drink, their body doesn't utilize it properly.

Sodium and potassium in the body are very important and besides controlling water in the body they also help with the voltage in the nerves and outside the nerves.Hypovolemia is related to sodium (salt) depletion which causes loss of water inside the blood vessels and is different from dehydration, which is excessive loss of body water.This basically means that if you aren't adding extra salt to your diet, it won't matter how much water you drink. If for some reason your kidneys aren't releasing ADH (anti-diuretic hormone) then how much water you drink isn't going to help either.

      "Patients should not drink excessive amounts of water because doing so can cause
       essential electrolytes to become diluted in the bloodstream, which may affect heart
       rhythm." POTS: What Helps

You can also find some basic information on how salt and your kidneys affect how your body uses water, despite how much you drink. If you drink more water than your kidneys can handle and the sodium in your body gets diluted it is called hyponatremia. The recommended amount of water
is about 64 oz. or about 2 liters. For men. the amount is higher, about 3 liters. That's about eight 8 oz. cups(13 for men). Preventing and treating orthostatic hypotension: As easy as A, B, C

You might want to drink more if you are in a hot climate or exercising. Then you could drink about twice that if you are a man. It's right after he tells people how much water they should be drinking and how much salt they should consume that he says there shouldn't be one guideline for anyone but different amounts for different people. This is kind of amusing considering for the rest of the video he is basically telling everyone to do the same thing. Even though he says that no two people will need the exact same spinal adjustment, he is still implying that everyone with POTS regardless of cause needs their spine adjusted.

Adrenal fatigue, he indicates that it is only caused by stress. It isn't even a proven medical condition. For those that believe in it, it is hypothesized that in the early stages there is too much adrenaline due to stress and then later not enough adrenaline. The theory is that the adrenaline is just slightly
low and that blood tests aren't sensitive enough to show it. But actual adrenal insufficiency can be found by tests. It is also known as Addison's disease. And that isn't caused by stress. It is an autoimmune disease. You can't cure that by reducing stress.

He says this unrecognized condition of adrenal fatigue causes autoimmune disease. But Addison's
disease an autoimmune disease itself and is often found with other autoimmune diseases like thyroid disease. To put that another way: he is saying that adrenal fatigue causes Addison's disease. Now how in the world does having a slightly lower than normal adrenaline level, (because that is what adrenal fatigue is) cause an autoimmune disease? All of the other symptoms he lists are caused by autoimmune diseases too.

POTS is often misdiagnosed as anxiety/stress  or as this guy indicated it is said to be the cause of
it. It is true that POTS and anxiety are associated with over-activation of the sympathetic nervous system. But POTS happens because of things that are activated by standing or physical activity. But anxiety/stress can occur regardless of  your body position. While he is talking about hyperadrenergic POTS and says that it can be caused by impaired clearance or decreased uptake by the synaptic cleft, he then turns around and says too much adrenaline is caused by physical, chemical or emotional stressors. But he just said that a Postgraduate Medical Journal said it was caused by not being reabsorbed like it should not by too much being released. One type of hyperadrenergic POTS is caused by a tumor called pheochromocytoma. That is most definitely not caused by a chronically stressed state.

He points out that he did a search for vaccines that cause POTS and got 9,440 results, allowing the viewer and his audience to believe that there are other vaccines that cause POTS. And he says not to  take any vaccines even though HPV/Gardasil is the only one that he points out as causing POTS. If you do a search, that is probably the only one you are going to find either. And only 3 states mandate HPV vaccine for school, but he says they all do. He also says that boys have to take it even though it's just to prevent cervical cancer. It also isn't a vaccine for cervical cancer. It is for genital herpes, which can cause cervical cancer and genital cancer in boys and anal cancer in both. Besides preventing genital cancer in boys, the idea is to stop the spread of it because if a boy gives it to a girl she can then get cancer and so can he. I'm not recommending the vaccine, just pointing out how inaccurate the things he says are.

As it turns out he was wrong even about Gardasil causing POTS. So, it doesn't matter what anyone turns up in a search because the CDC determined that it doesn't and the old links are now outdated. "In November 2015, the European Medicine’s Agency completed a detailed review of available POTS data from young women who received HPV vaccines.  The review found that the evidence does not support a causal link between HPV vaccines and POTS." Centers For Disease Control:Frequently Asked Questions about HPV Vaccine Safety I don't know when he made the video, but it was just uploaded a few days ago. So the information was out there for him almost a year ago.

Antibiotics: he names them off as bad too. What do you do if you have an autoimmune disease that causes your POTS and you are on an immunosuppressant and get an infection?

He also says not to use sunscreen and to use coconut oil. Stop Risking Skin Cancer! Coconut Oil Is No Sunscreen.

"Neither coconut oil nor any of the other oils will protect your
skin from the sun's ultraviolet rays so you will need to apply
sunscreen when going outdoors, particularly in sunny weather."
http://www.webmd.boots.com/healthy-skin/guide/coconut-oil-as-a-beauty-product

It seems like much of what he says is unfounded information. He could be correct about some things, but I wasn't able to find sources that backed him up, and it was a lot easier to find sources that contradicted him.

He recommends really high doses of vitamin D.(8000 IU per day).

Mayo Clinic: Drugs and Supplements Vitamin D
National Institutes of Health 
(They recommend no more than 4000, but optimally about 1000)
The vitamin D council say about 5000 IU. And it's their business.
https://www.vitamindcouncil.org/blog/why-does-the-vitamin-d-council-recommend-5000-iuday/
National Osteoporosis Foundation 1000 mg https://www.nof.org/patients/treatment/calciumvitamin-d/
Institute of Medicine (IOM), the safe upper limit of vitamin D is 4,000 IU per day for most adults.
But they recommend about 800IU for normal people.
Vitamin D Supplementation
You can get pretty sick if you take too much vitamin D.Mayo Clinic: Vitamin D Toxicity

Then he says chiropractic adjustment takes pressure off of the nervous system. But there is much more to the nervous system than just the nerves.

There are even three different parts to the nervous system, the central(CNS), peripheral(PNS), and autonomic nervous systems(ANS). You can think of it like this: The CNS sends commands out to the PNS. The PNS has receptors that are affected by chemicals and they send information back to the CNS which is the brain and spine.

Sometimes information goes straight to the brain so that the brain can decide what response to have. Other times, information goes to the spine and the spine basically makes the decision before it ever gets to the brain. This is called the reflex loop.

If this was what always happened, then it would make sense for spinal manipulation to work. But it isn't. You can work on the spine all you want, but it won't either make the brain work better or have any effect on those receptors on the other end of the PNS.

There are lots of things that can interfere with this communication system. For instance, the myelin sheath on nerves can be damaged. This happens with Multiple Sclerosis or a severe electrical shock or lightning strike. Myelin serves the same purpose as the plastic coating on an electrical wire. It keeps the electrical charge from leaking into the surrounding tissue. If it's damaged, the signals can fade or disappear. This is called demyelination. They don't get where they were supposed to be going.

He briefly touched on the parasympathetic and sympathetic nervous systems, which are the chemicals switching on some things and switching off some things. Those fight or flight hormones are also
countered by rest and digest hormones that control things like your digestive system.It cannot be helped by working on your spine.

The autonomic nervous system could also be called the automatic nervous system and it is controlled by your brain stem. It controls things like your internal organs. He refers to a journal that calls those organs your viscera.  You would be well advised not to mess with your brain stem by doing some kind of adjustment, particularly in an effort to effect the heart. When he quotes a journal saying that it(manipulating the spine to affect the heart) MAY work, he is quoting a chiropractic journal(Journal of Manipulative and Physiological Therapeutics) and not a medical journal. If you look that journal up it says that this is at odds with classic views of neuroscientists about the potential for somatic stimulation of spinal structures to affect visceral function. http://www.jmptonline.org/article/S0161-4754(00)90076-9/abstract Why do you suppose he chose to leave out that neuroscientists don't agree with this theory?

When he starts talking about the sympathetic nervous system being located in the thoracic spine, that is true. But when it sends out signals, it also needs those chemicals/neurotransmitters like acetylcholine and norepinephrine sometimes called noradrenaline.

Part of the parasympathetic nervous system originates in the sacrum/lower spine and some of it up in the brain stem area called the medulla. It also requires acetylcholine. He says that if you work on one of these areas of the spine it will change the nerve supply and function of the organs.He seems to be talking about the sympathetic area of the spine and forgetting that the parasympathetic system
even plays a part. The reason I say this is because he refers to the thoracic area of the spine and
keeps talking about the "fight or flight" response and stress. But some people with dysautonomia
have problems with the parasympathetic nervous system. If you think of the sympathetic nervous
system as the ON switch and the parasympathetic as the OFF switch. A problem can be just as much a problem of the OFF switch not working as it can be of the ON switch working too much. The sympathetic nerves originate in the spinal area; they are fairly short and close to the organs they stimulate (a few inches in length). When they switch on, they do so quickly and the entire system is "on." The parasympathetic nerves, on the other hand, originate mostly from the brain, but some originate from the bottom of the spine, so they travel much longer distances to the organs which they stimulate (several yards); they are much slower to react and because of this it takes a much longer time for the body's systems to  "turn off." You can easily see from this explanation that because some of these nerves originate in the brain, working on the spine won't fix problems with how they work.

 If you think of this like a telegraph system, then he is talking about working on the telegraph machine itself. But then you still have the wires themselves as well as the telegraph machine on the other end that he isn't taking into account. Then you have to factor in the chemicals and hormones which are basically the electricity running through that wire.

He points out exercise as being a treatment for POTS. The problem is it doesn't work for everyone.
They have also done studies that show why it doesn't work. It is coauthored by Svetlana Blitshteyn
She is another expert on POTS.
       "This study provides the first objective evidence that low ventricular filling pressures
        in patients with POTS are contrary to what would be expected in deconditioned
        patients—high filling pressures.Although the benefits of exercise have been acknow-
        ledged in several studies, almost 60% of patients with POTS are unable to complete
        an exercise training program despite their efforts. Importantly, Oldham et al.
       demonstrate that exercise intolerance in POTS is not caused by a lack of maximum
       effort from the patient but that low ventricular pressures occur despite the maximum
       effort."  Pulmonary Vascular Research Institute.

I can entirely understand not wanting to take medications that might have side effects.
But it is bad medicine to say that it is a viable option for everyone. If you keep an open mind and really want to understand how your nervous system works and watch these videos, you can learn. And once you do, it probably won't seem so much like what he says makes any sense. Just because he is recommending not taking medicine,which works for some patients, doesn't mean that everything else he says makes sense.

You will see that there is much more involved than just the spine. He way oversimplified it. For instance, spinal manipulation might help if you have bowel problems related to your POTS causing a balancing effect on the nerves that supply impulses to the intestinal tract.

 But what about that serotonin that originates in the gut or other neurotransmitters? If those are out of balance, you may still have problems. That doesn't even factor in the enteric nervous system. It works like a secondary brain and has structures and chemicals similar to those in the brain. It has sensory and motor neurons and information processing circuits, as well as glial cells. It uses neurotransmitters: dopamine, serotonin, acetylcholine, nitric oxide, and norepinephrine. It even has benzodiazepines, similar to Valium and Xanax.


The Nervous System - CrashCourse Biology #26


Autonomic Nervous System: Crash Course A&P #13(This one explains how the parasympathetic
and sympathetic originate in different areas of the body)





Sympathetic Nervous System: Crash Course A&P #14 This one explains how hormones and neurotransmitters are necessary to the system. And they have nothing to do with chiropractics. And quite a bit to do with nerve receptors. If you pay close attention it touches on alpha and beta receptors, which are why beta blockers are used. And the adrenal glands, which if there is something wrong with them, cannot be fixed by spinal manipulation or drinking water.



Parasympathetic Nervous System: Crash Course A&P #15 This one explains how your parasympathetic system is supposed to keep your heart rate down. Remember that it doesn't originate in the thoracic area that this guy keeps referring to. And many of them don't even run through the spine. Check out the vagus nerve which goes from the brain directly to the heart, lungs, and stomach. No spine involved.

Peripheral Nervous System: Crash Course A&P #12  The peripheral nervous system doesn't start at the spine. It starts on the other end. And if the receptors, etc. on that end don't work right, they aren't going to send information back to the spine the way they should. And even though the information  goes through the spine, ultimately it should end up in the brain. And the brain should affect the response.


These articles have fairly comprehensive lists of treatments for POTS. It starts out with the
homeopathic or nonpharmaceutical treatments, which common sense tells you should be tried first. http://myheart.net/pots-syndrome/treatments/
British Journal of Cardiology Postural Orthostatic Tachycardia Syndrome (POTS): A Diagnostic Dilemma


I gave a lot of sources because at the end of his video he gave some sources. The first page
and a half of them are about side effects of medicines that I have shown are not always given for POTS and many of which aren't recommended by doctors who specialize in POTS, like cardiologists and neurologists.

Chiropractic manipulation has its place. It eases people's pain and that will help their overall health but in my opinion, it doesn't seem to be good science to say that it can do the things he claims. 

Wednesday, September 21, 2016

Morbidity--Can You Die From POTS or Associated Conditions?

This post is going to be a hard one for me to write and probably a hard one for some people to read. The possibility or your own mortality is a subject most people try not to think about.

The reason I am writing this is because I had some people get angry at me in a facebook group for suggesting that you could die from POTS.

While it is true that POTS itself doesn't kill people, there is a series of possible events that can ultimately result in death. And I know it to be a fact because my husband died from it. This is what happens:

Hypotension>hypoperfusion>syncope>seizure>cardiac event.
 And it can progress quickly.
My husband went outside, got too hot came in and sat down and a few minutes later, about five, he was in cardiac arrest. And he was never revived by either the EMTs or the ER doctors. Officially, his death certificate says cardiac arrest. But he passed out, had a seizure and then his heart stopped.

I am going to try and explain as well as I can how that is possible.

MASTOCYTOSIS
Here is an article about a man who had a heart attack because he had mastocytosis, which caused him to have syncope, and then the heart attack. Shortness of breath, syncope, and cardiac arrest caused by systemic mastocytosis

Mast cells secrete histamines that cause rashes and hives in an allergic reaction. They also play a role in wound healing and in the immune system.

When someone's mast cells cause them problems, they can have abdominal pain, cramping, diarrhea, flushing, itching, wheezing, coughing, lightheadedness and potential problems with “brain fog” or other difficulties with memory. And there are some patients who have POTS and MCAS/Mast Cell Activation Syndrome. You can read more about POTS and MCAS here: A Tale of Two Syndromes – POTS and MCAS Mastocytosis is when too many of those mast cells remain in the tissues of the body.

 So there is one way POTS can cause a cardiac event, even if it is indirectly because of MCAS.

“Syncope is defined as a sudden loss of consciousness with loss of stature. It is due to the sudden decrease of oxygenated blood perfusion to the brain. If the cause of cerebral hypoperfusion resolves within 1-2 minutes, the consciousness returns. It is not uncommon to observe convulsions during the episode of syncope, which is attributed to the sudden lack of oxygen and glucose to neurons resulting in disorganized neuronal activity. If the cause of cerebral hypoperfusion persists and the individual does not get prompt medical help with cardiopulmonary resuscitation (CPR), permanent damage may occur.” https://www.hawaii.edu/medicine/pediatrics/pedtext/s07c08.html

“It is not uncommon that due to the loss of cerebral perfusion, convulsions can occur for a few seconds or even up to a minute.”

So this says that regardless of what causes the syncope if you don't have prompt medical attention it can progress to the point of needing CPR and permanent damage. Seizures can actually last longer than it says above. “Although usually brief, the convulsions associated with syncope can be quite severe and prolonged.” http://onlinelibrary.wiley.com/doi/10.1111/j.1528-1157.1997.tb01086.x/pdf

Convulsions or seizures in relation to syncope are complicated. Seizure activity in the brain can cause hypotension and syncope. But hypotension and syncope can cause seizures. “While neurally mediated syncope may mimic seizure-like activity, it should also be acknowledged that seizure foci in certain cerebral sites (particularly the temporal lobe) may be the source of apparent neurally mediated syncopal events. Localized seizure activity may initiate the reflex arc previously described, leading to hypotension and bradycardia.” The pathophysiology of common causes of syncope

Bradycardia can happen as a result of a seizure. http://www.seizure-journal.com/article/S1059-1311(14)00070-3/fulltext



"Convulsions suggest prolonged or severe brain hypoperfusion. Shakiness, which can accompany hyperadrenergic activity, can simulate seizure. The duration of loss of consciousness as well as the position of the patient during loss of consciousness is important information. Urinary incontinence and tongue biting during a spell favor a seizure event.

Helping the patient sit or lie down quickly and raising the legs above the heart level permit faster recovery in patients with a typical reflex postural hypotension event. Physicians should check the pulse for amplitude and rhythm. When a patient recovers the acute event, ambulation should be resumed with care because recurrence of hypotension may be inevitable at this stage due to circulatory instability. Oral hydration with salty fluids usually is helpful in the early recovery phase if the patient has no known previous history of heart disease. Serious arrhythmogenic events, coronary insufficiency syndromes, pulmonary embolism, strokes or transient ischemic attacks, and blood loss must be recognized for proper immediate medical care. Injuries sustained during a sudden fall require immediate attention." Cleveland Clinic--Syncope

Asystole is a cardiac arrest rhythm in which there is no discernible electrical activity on the ECG monitor. Asystole is sometimes referred to as a “flat line.”

SUDEP sudden unexpected death in epilepsy.

Apnea is when you stop breathing.

“A principal finding of this study was that apnea and asystole occurred in all SUDEP cases in the early postictal phase after generalized tonic–clonic seizures.” The same article says that usually the asystole or flat-line stops when hypoperfusion and anoxia return to normal. This just means that during a seizure blood flow and oxygen are being interupted but for some people it doesn't return to normal and they die.

“ In conclusion, this patient most probably presented TLOC(total loss of consciousness) episodes due to both reflex syncope and temporal lobe seizures (without a clearly proven link between seizures and syncope).” So you can have both syncope and seizures. The article also says that if seizures are causing the asystole/heart stopping, that a pacemaker is a good treatment option. It was written in 2014. And my husband never lived long enough for us to learn about this. http://www.seizure-journal.com/article/S1059-1311(14)00070-3/fulltext I found a cardiology journal that says the same thing. “Ictal bradycardia/asystole syndrome is mostly related to temporal lobe epilepsy, predominantly in male patients aged older than 50 [44–49]. The diagnosis is based on simultaneous EEG and electrocardiography (ECG) recording with electrographic seizure activity preceding severe bradycardia/asystole or AV-block. A combination of antiepileptic treatment and pacemaker therapy might be required in such patients.” Is it possible to accurately differentiate neurocardiogenic syncope from epilepsy?

“Although convulsive syncope or anoxic seizures are not uncommon, actual epileptic seizures induced by vasovagalor similar forms of syncope have only rarely been reported.” RARE BUT IT DOES HAPPEN. “About 30 s elapsed between the syncope and the onset of the seizure discharge.”
“Of 13 previously reported patients with epileptic seizures induced by syncope, only one had a history of epilepsy with an abnormal interictal EEG.” “it has been speculated by some that a predisposition to generalized epilepsy exists in these patients and that the hypoxic or ischemic syncopal episode might further lower the seizure threshold, resulting in the observed epileptic seizures in these susceptible patients.” http://onlinelibrary.wiley.com/doi/10.1111/j.1528-1157.1997.tb01086.x/pdf

hypotension >Bradycardia>hypoperfusion>sycope>seizure>apnea and asystole/flat-line
seizure>hypotension and bradycardia>hypoperfusion>syncope
That makes two.

Given that one can cause the other, it could be a vicious cycle.


This is another scenario.


This medical paper says that orthostatic hypotension and bradycardia can cause syncope and Atrial fibrillation. “ This is the first report of a patient with persistent atrial fibrillation associated with syncope caused by orthostatic hypotension and bradycardia.” Persistent atrial fibrillation associated with syncope due to orthostatic hypotension: a case report.

This is another article that says that a man had a tilt table test where they induced Afib and that caused him to have syncope. And the next day they did a tilt table test and he had syncope that caused Afib.New England Journal of Medicine--Neurally Mediated Syncope and Atrial Fibrillation


And Atrial Fibrillation can cause sudden cardiac death. “Incident AF is associated with an increased risk of SCD.” Atrial fibrillation and the risk of sudden cardiac death: the atherosclerosis risk in communities study and cardiovascular health study.


hypotension>bradycardia>hypoperfusion>syncope>Afib>Sudden Cardiac Death

And one of the symptoms of POTS is syncope. So, therefore, it follows that the T in POTS or tachycardia causes syncope and therefore can cause Afib and Sudden Cardiac Death.

“The postural tachycardia syndrome (POTS) also presents clinically with symptoms of cerebral hypoperfusion.” Orthostatic Hypertension: When Pressor Reflexes Overcompensate


“This supports the hypothesis that repetitive stable VT(ventricular tachycardia) can play a role in the pathophysiology of cerebrovascular insufficiency.”


“cardiac syncope a variable amount of hemodynamic instability results in cerebral hypoperfusion”

“Systemic hypotension is a common postoperative complication that can cause hypoperfusion and inadequate delivery of oxygen and substrates to organ systems The systemic blood pressure at which the risk of complication increases depends in part on the preoperative blood pressure.”POSTOPERATIVE HYPOTENSION  What this article is getting at is that POTS and orthostatic hypotension patients can go into shock after surgery easier than other people. 

hypotension>hypoperfusion>

Hypoperfusion>syncope
“ These findings suggest that cerebral hypoperfusion, such as with cerebral vasospasms, before the onset of bradycardia might be involved in the mechanism of neurally mediated syncope in patients with an aura.” Possible involvement of cerebral hypoperfusion as trigger of neurally-mediated vasovagal syncope.


syncope>hypoperfusion

hypotension>Afib

“The study suggests that a bout of orthostatic hypotension — a steep blood pressure drop that occurs when a person stands up after a period of lying down — appears to be associated with an overall 40 percent increase in the risk of developing atrial fibrillation over the following two decades.”

Although the above links are referring to orthostatic tachycardia, POTS also is associated with hypoperfusion. Decreased upright cerebral blood flow and cerebral autoregulation in normocapnic postural tachycardia syndrome

"POTS manifests with symptoms of cerebral hypoperfusion and excessive sympathoexcitation.


"The mechanism of POTS is still undergoing a lot of investigation and may be multifactorial. Therefore, any possible overlap with atrial fibrillation is still somewhat unclear and may warrant further study."

Tachycardia>syncope>Afib>Sudden Cardiac Death

It also isn't a far stretch to say:

Tachycardia>syncope>seizure>apnea and asystole/flat-line

Here Is Another Scenario


An enlarged heart isn’t a condition in itself, but a symptom of an underlying problem that is causing the heart to work harder than normal.

“However, if you experience unpleasant symptoms or a permanently increased heart rate is risking heart enlargement, your doctor may recommend treatment with medication or catheter ablation.”Supraventricular Tachycardia

“Tachycardia-induced cardiomyopathy develops slowly and appears reversible by left ventricular ejection fraction improvement, but recurrent tachycardia causes rapid decline in left ventricular function and development of heart failure. Sudden death is possible.” Cardiomyopathy is when the heart muscle becomes enlarged, thick, or rigid. AHA JOURNAL--Heart Failure and Sudden Death in Patients With Tachycardia-Induced Cardiomyopathy and Recurrent Tachycardia

“Some forms of enlarged heart can lead to disruptions in your heart's beating rhythm. Heart rhythms too slow to move blood or too fast to allow the heart to beat properly can result in fainting or, in some cases, cardiac arrest or sudden death.” Mayo Clinic--Complications of Enlarged Heart

So to sum up, tachycardia can cause an enlarged heart which can cause cardiac arrest or sudden death.


A patient can have both seizures and syncope.

Seizures>bradycardia/asystole or AV-block

hypotension >Bradycardia>hypoperfusion>sycope>seizure>apnea and asystole/flat-line SUDEP

seizure>hypotension and bradycardia>hypoperfusion>syncope

orthostatic hypotension and bradycardia can cause syncope and Atrial fibrillation.

hypotension>bradycardia>hypoperfusion>syncope>Afib>Sudden Cardiac Death

Afib>syncope

Tachycardia>syncope>Afib>Sudden Cardiac Death

Tachycardia>hypoperfusion>

Hypoperfusion>bradycaria>syncope

Tachycardia>syncope>seizure>apnea and asystole/flat-line


If those aren't enough for you, here are some more. 


"There are also a number of symptoms and signs that may indicate that a person is at increased risk for SCD. These include:
  • An abnormal heart rate or rhythm (arrhythmia) of unknown cause
  • An unusually rapid heart rate (tachycardia) that comes and goes, even when the person is at rest
  • Episodes of fainting (called syncope) of unknown cause"

RISKS DURING PREGNANCY

There has been debate over whether or not POTS patients should opt for a caesarean section or a vaginal birth.  Due to the physical stress of a vaginal birth, some patients have gone into cardiac arrest (or near cardiac arrest) while delivering.  The reason to opt for a C-section would be to avoid this incidence.  However, a C-section is major surgery and must be discussed thoroughly with a high-risk obstetrician.   Also, some patients may have problems receiving anesthesia which is administered during a C-section and sometimes during vaginal birth (also known as an epidural).  Both of these aspects must be thoroughly reviewed prior to delivery.How does POTS affect pregnancy and labor?



PROBLEMS DURING HEAD UP TILT TABLE TEST
HUT testing is generally safe, but there have been occasional reports of coronary vasospasm, chest pain, hypertensive crisis and tachyarrhythmia. The most frequent adverse effects are hemodynamic changes, such as hypotension, tachycardia or bradycardia associated with orthostatic intolerance, presyncope or syncope. It is noteworthy that patients with neurocardiogenic syncope may rarely experience asystole (defined as ventricular pause of more than 5 seconds) or complete atrioventricular block during HUT testing. Lacroix and colleagues reported 10 asystolic reactions (6%) (average duration 12 seconds) among 179 patients investigated for neurocardiogenic syncope; 8 patients needed cardiopulmonary resuscitation for 20 to 30 seconds. Dhala and associates reported 19 asystolic reactions (9%) among 209 patients with suspected neurocardiogenic syncope and 3 asystolic responses (4%) among 75 healthy control subjects during HUT testing without pharmacologic stimulation. These subjects did not show a worse outcome than their nonasystolic counterparts during follow-up. The fainting patient: value of the head-upright tilt-table test in adult patients with orthostatic intolerance


Autonomic nerve disorders (dysautonomia) refer to disorders of autonomic nervous system (ANS) function. Dysautonomia is a general term used to describe a breakdown or abnormal function of the ANS. 

POTS and Orthostatic hypotension are dysautonomias.

About half of patients with POTS have a restricted autonomic neuropathy with a length-dependent distribution of neuropathy.

"While diabetes is generally the most common cause of autonomic neuropathy, other health conditions — even an infection — may be to blame." http://www.mayoclinic.org/diseases-conditions/autonomic-neuropathy/basics/definition/con-20029053


(This is associated with diabetes but it is possible that other people could have it: see above.)

"Cardiac autonomic neuropathy (CAN) represents a serious complication as it carries an approximately
five-fold risk of mortality in patients with diabetes just as in those with chronic liver diseases. The high
mortality rate may be related to silent myocardial infarction, cardiac arrhythmias, cardiovascular and
cardiorespiratory instability and to other causes not yet explained. Resting tachycardia due to parasympathetic damage may represent one of the earliest signs. Typical findings referring to autonomic dysfunction may include exercise intolerance, orthostatic hypotension
and cardiac dysfunction to rest or exercise. Severe autonomic neuropathy may be responsible for
spontaneous respiratory arrest and unexplained sudden death." Autonomic neuropathy: a marker of cardiovascular risk


"Resting tachycardia due to parasympathetic damage may represent one of the earliest signs of CAN. Experiences from large epidemiological studies indicate that tachycardia of any origin is a major risk factor for cardiovascular and non-cardiovascular death.17 The heart rate-mortality association is observed at any age."

"CAN is associated with a high risk of unexpected and sudden death, possibly related to silent myocardial ischaemia/infarction, cardiac arrhythmias and hypoxia.15 Cardiorespiratory arrests during
or right after anaesthesia have been described."

. Possible factors associated with high mortality and sudden death due to autonomic neuropathy 
● Silent myocardial ischaemia/infarction 
● Cardiorespiratory arrest/increased perioperative and peri-intubation risk 
● Resting tachycardia 
● Ventricular arrhythmias/prolongation of the QT interval 
● Hypertension 
● Orthostatic hypotension 
● Flattening of the nocturnal reduction of blood pressure and heart rate (‘non-dipper’ phenomenon) 
● Exaggerated blood pressure responses with supine position and exercise 
● Abnormal diastolic/systolic left ventricular function 
● Poor exercise tolerance 
● Impaired cardiovascular responsiveness 
● Heat intolerance due to defective sympathetic thermoregulation 
● Susceptibility to foot ulcers and amputations due to arteriovenous shunting and sudomotor dysfunction 
● Hypoglycaemia unawareness (?) 
● Increased risk of severe hypoglycaemia 
● Obstructive sleep apnoea syndrome

Autonomic neuropathy is common in Nigerian patients with non-diabetic Chronic Renal Failure.http://www.ncbi.nlm.nih.gov/pubmed/15171518

Renal Failure isn't something that is associated with POTS but autonomic neuropathy is. 
"The role of obesity is supported by the high prevalence of cardiac autonomic dysfunction in non-diabetic obese people,"Diabetic Cardiovascular Autonomic Neuropathy

"Diabetes can produce the symptoms of POTS (Llamas, Garcia, Gaos, Jimenez, Villavicencio, Cueto & Arriaga, 1985). There are different types of diabetes, including diabetes insipidus, that are associated with POTS symptoms"http://www.dinet.org/index.php/information-resources/pots-place/pots-causes

"POTS is also often classified as primary or secondary.  An example of an 2003 classification of postural tachycardia syndrome by Dr. Grubb (18):

Primary forms: Partial dysautonomic, Immune mediated pathogenesis, Adolescence, Hyperadrenergic state

Secondary forms: Diabetes mellitus, Amyloidosis, Heavy metal poisoning, Sjogren syndrome, Hypermobility syndrome, Paraneoplastic syndrome"

"Both Peripheral (PN), and specifically Small Fiber Neuropathy (known as SFN, a type of PN), have been associated with Autonomic Neuropathy, POTS, and other diseases that cause POTS, such as Diabetes and Sjogrens" what-is-causing-your-pots-and-why-it-is


You can read more on autonomic neuropathy here: Autonomic Neuropathy



Sudden Cardiac Death


Autonomic Neuropathy Causes 2.5 Times a Risk of Sudden Death













Sunday, September 18, 2016

What Is Dysautonomia?

Autonomic Neuropathy Treatment & Management


Autonomic Neuropathy Treatment & Management--Medical Paper

If You go to the above link and create a sign in password this is a great medical paper/article. 




Blood Tests and Labs for Dysautonomia





Lest you think I suddenly became really smart, I got this information from the following: Medscape: Autonomic Neuropathy Workup, Medical Paper

You need to have a complete blood count, basic metabolic panel, liver function testing, and immunoelectrophoresis. More specific testing should be based on the patient’s history of other medical conditions.

Special situations

Depending on what the results of the first blood tests and autonomic tests are, there are more tests that may be ran.


Oral glucose tolerance test to check for diabetes mellitus, if an initial serum glucose level is normal or nondiagnostic.

They can test for SS-A and SS-B if they think you may have Sjögren's syndrome.
Anti-ganglionic acetylcholine receptor (AChR) autoantibodies if the onset was acute to subacute in nature.

Specific genetic tests for the familial dysautonomia can be done.

Specific tests for infections, inflammatory, autoimmune, and paraneoplastic causes can be ordered based upon the history and physical examination.

Measurement of basal plasma norepinephrine levels can be useful in specific forms of autonomic neuropathy. In pandysautonomia, basal norepinephrine levels are low and do not rise on head-up tilt table testing. Following an overnight supine position, low norepinephrine levels can be found in patients with POTS.

A history of neuropathy, mental status changes, and abdominal pain should prompt the physician to evaluate the patient for acute intermittent porphyria. In cases of suspected porphyria, high levels of porphobilinogen and delta-aminolevulinic acid can be found in urine during acute episodes.
Evaluation of cerebrospinal fluid (CSF) via lumbar puncture can be useful in specific cases.

In pandysautonomia, CSF protein is elevated, as is CSF enolase, which may indicate damage to the dorsal root ganglia. (Acute pandysautonomia is a rare disease defined as acute widespread and severe sympathetic and parasympathetic failure and sparing of somatic nerve fibers. The causes of this syndrome are often an autoimmune disease leading to autonomic ganglionopathy.)

In HIV or AIDS, the CSF may demonstrate an elevated protein as well as pleocytosis.

Paraneoplastic varieties of autonomic neuropathies also tend to show an inflammatory picture in the CSF. However, abnormal CSF protein is not specific for autoimmune, inflammatory, or infectious causes of autonomic neuropathy.

Imaging tests

In addition to CT and MRIs,

SPECT and PET scanning may identify cardiac sympathetic dysfunction in both type I and type II diabetes mellitus.

The pattern of sympathetic disturbances tends to be heterogeneous, with denervation affecting mainly the posterior myocardial region, whereas focal hyperinnervation can be observed of the proximal segment.

Autonomic testing

Autonomic testing  should be done to find out which part of the autonomic nervous system is effected and how severely.

Tilt table testing to test adrenergic vasomotor function and cardiac sympathetic function.

Cardiac response to deep breathing and R-R interval to evaluate cardiovagal functions.

Cardiac response to Valsalva maneuvers to test parasympathetic innervation to the heart.

Quantitative Sudomotor Axon Reflex Testing (QSART) to evaluate the postganglionic segment of the thermoregulatory pathway. Four regions are tested: forearm, proximal leg, distal leg, and dorsum of the foot. Electrical stimulation (iontophoresis) is applied to the skin, and the volume of sweat produced can be measured.

Nerve conductions studies and electromyography
Findings on nerve conduction studies (NCS) and electromyography (EMG) can be normal in pure autonomic neuropathies because the involved fibers are small myelinated and unmyelinated fibers, which cannot be assessed with NCS or EMG.

Findings on nerve conduction studies (NCS) and electromyography (EMG) can be normal in pure autonomic neuropathies because the involved fibers are small myelinated and unmyelinated fibers, which cannot be assessed with NCS or EMG.

In autonomic neuropathies with concomitant sensory neuropathy, absence of sensory potentials may occur.

In autonomic neuropathies with concomitant sensorimotor neuropathy, marked loss of motor and sensory potentials is noted.

In cases of suspected neuromuscular transmission defect, such as with botulism or LEMS, a typical electrophysiologic pattern of low-amplitude compound muscle action potentials increasing with high-frequency repetitive stimulation is characteristic of a presynaptic neuromuscular defect.

Specialized studies

In Sjögren syndrome, results of the Schirmer test with a rose-Bengal eye stain, as well as lip biopsy to identify chronic sialoadenitis, can be diagnostic.

Postprandial blood pressures: An abnormal result would be to measure a drop in systolic blood pressure of >20 mm Hg approximately 15-20 minutes after a meal.

Other uncommon bedside stimuli that can be used to assess for a rise in blood pressure during continuous blood pressure monitoring include isometric exercise (sustained hand grip for 3 min), a cold pressor test (immersion of a hand in ice water for 90 s), and mental arithmetic (with serial-7 or serial-17 subtraction), all of which stimulate sympathetic outflow and elevate blood pressure in healthy patients.

Multiple daily blood pressures to examine for diurnal fluctuation: A difference of < 15 mm Hg with either systolic or diastolic blood pressure between daytime (awake) values and nighttime (sleeping) values could indicate presence of autonomic neuropathy.

Specific autonomic tests that are being performed at some institutions include the following:

The thermoregulatory sweat test (TST) complements the quantitative sudomotor axon reflex test (QSART) and is used to assess thermoregulatory pathways. The patient is covered with alizarin red powder, which, when moist, changes from orange to purple. The patient's temperature is then raised above core temperature, and photography is performed to map for areas of color change, revealing areas of anhidrosis/hypohidrosis where color did not change. The TST and QSART can both be useful in idiopathic anhidrosis. A lack of color changes with the TST is essentially diagnostic for postganglionic sudomotor dysfunction.

Sympathetic skin responses (SSR) can be assessed with routine EMG equipment. This test can be used to identify indirect evidence of sweat production via measurement of changes in skin conductance on the palm/sole in response to an electrical stimulus. The stimulation of an afferent somatic branch with SSRs gives an assessment of potential adrenergic sweat production. Brief electrical stimuli are administered at intermittent intervals and a response is measured from the hands or the feet, representing a change in skin resistance due to sweating.

Quantitative sensory testing (QST) can be helpful in autonomic disorders with sensory neuropathy. QST permits comparison of sensory thresholds by using vibration and temperature perception to assess both large and small-fiber modalities. These patients typically have impaired thresholds for heat and pain[54] , but vibration and cool sensitivity may be normal.

Pupillometry measure changes in papillary response and is being investigated at some institutions as a potential marker for autonomic neuropathy.
Quantitative direct and indirect test of sudomotor function (QDIRT) involves making a silicone impression of a patient's skin while sweating is induced by acetylcholine iontophoresis. The presence of sweat droplets can be quantified in the silicone cast, providing a marker of sudomotor function.
Vascular studies are occasionally useful in assessing autonomic neuropathy.


Vascular studies are occasionally useful in assessing autonomic neuropathy.

Adrenergic function can be assessed by measuring skin blood flow, transcutaneous oxygenation, and skin temperature.

Doppler probes can be used for blood flow measurements.

Infrared thermometry and telethermography can be used to measure skin temperature.

Assessment of skin temperature can be useful in patients with small-fiber neuropathy.

Urological studies

Urodynamic studies may be used to examine the lower urinary tract function.

Measurements include urine flow rate, residual volume, cystometry during filling and voiding, urethral pressure profile measurements, and pelvic floor neurophysiology.

An important measure in assessment of a neurogenic bladder is the postmicturition residual volume; this can be measured invasively by urethral catheterization after voiding, but it can also be measured noninvasively with ultrasonography.

Gastrointestinal studies

Videofluoroscopy is useful in assessment of swallowing in the presence of oropharyngeal dysphagia.
A barium swallow study, meal, and follow-through study are helpful in suspected upper gastrointestinal disorders, though endoscopic assessment provides the opportunity for biopsy in particular situations, as well as better visualization.

Esophageal manometry may be of value in disorders of motility and esophagogastric function.
Gastric motility may be assessed by using radioisotope methods and scintigraphic scanning.
In cases of small-bowel disorders suspected to be neurologic in nature, manometry may be of value in discriminating myopathic from neuropathic disorders. Large-bowel dysfunction can be assessed via measurement of transit time.

Esophageal manometry and gastric emptying scintigraphy can also be useful in patients with possible autonomic neuropathy and dysphagia.

Diabetic patients with symptoms of esophageal dysmotility have insufficient lower esophageal sphincter relaxation and a higher percentage of simultaneous waves detected, while diabetic patients with cardiovascular autonomic neuropathy have greater pathological simultaneous contractions.

Esophageal dysmotility and delayed gastric emptying may occur in up to 50% of diabetic patients. In particular, reports of abdominal fullness predicted delayed gastric emptying.

Biopsy findings

Sural nerve biopsy is occasionally diagnostic for types of autonomic neuropathy. In inherited autonomic neuropathies, a selective loss of particular fiber types can indicate the diagnosis. In autoimmune or infectious mediated forms of autonomic neuropathy, small perivascular infiltrates may be visible. In amyloidosis, characteristic Congo red staining indicates the presence of eosinophilic, extracellular, amorphous material surrounding perineurial and endoneurial vessels and within sympathetic ganglia and vagal nerves.

Epidermal skin biopsy can be used in the diagnosis of small-fiber neuropathies. This technique is less invasive than nerve biopsy. In autonomic neuropathies, autonomic fibers are deeper than the epidermal level; therefore, deeper biopsy is required to assess the fibers innervating sweat glands and piloerector muscles. In general, autonomic neuropathies of greater severity are associated with reduced epidermal fiber densities.

As distal endings are primarily involved in distal axonopathy forms of neuropathy, skin biopsy may be more sensitive than sural nerve biopsy to detect early abnormalities. Skin biopsy is also useful in congenital causes of autonomic neuropathy, as in congenital insensitivity to pain with anhidrosis (CIPA), where a lack of nerve fibers in the epidermis and only a few hypotrophic and uninnervated sweat glands are found in the dermis.

Immunologic Findings

Patients with autoimmune autonomic neuropathy can have antiganglionic acetylcholine receptor (AChR) autoantibodies.[66] Patients with high antibody values (>1.00 nmol/L) tend to have a constellation of sicca complex (marked dry eyes and dry mouth), abnormal pupillary light responses, upper gastrointestinal symptoms, and neurogenic bladder. Higher antibody titers correlate with greater autonomic dysfunction as well as increased frequency of cholinergic dysautonomia.

Patients with POTS may also demonstrate presence of ganglionic receptors.

In specific disorders, testing for the presence of autoantibodies can help determine a diagnosis. Antinuclear antibodies and antibodies to Sjögren's syndrome antigens A and B (SSA and SSB) are seen in several connective tissue disorders. Antibodies against voltage-gated calcium channels (VGCC) are associated with LEMS.

The combination of tilt table testing, cardiac responses to deep breathing and the Valsalva maneuver, and QSART comprise the composite autonomic scoring scale (CASS), which may be used to assess the severity of autonomic dysfunction. The CASS is reliable and useful for monitoring clinical progression with an autonomic neuropathy. The CASS is a 10-point scale; 4 points are allotted for adrenergic and 3 points each for sudomotor and cardiovagal failure. Scores are normalized for age and sex. Patients with a score of less than 4 on the CASS have mild autonomic failure; a score of 4-6 suggests moderate autonomic failure; and a score of 7-10 implies severe failure.

The TST can be useful in monitoring progression of idiopathic anhidrosis and Sjögren's syndrome where prominent anhidrosis/hypohidrosis occurs.

A Little Humor