Autonomic Failure & Orthostatic Hypotension

[Music] there we go so my task today was to talk about um one aspect of what paula introduced you to and that's the concept of autonomic failure and to try to put that into context with um other disorders you'll hear about over the course of the day and and to talk specifically about orthostatic hypotension or neurogenic orthostatic hypotension which is the main symptom you see in patients autonomic failure i do have some disclosures since this is a cme talk i've gotten some research support from dysautonomia international as well as from athena diagnostics i do some consulting work for for those folks and toward the end of the talk i'll have to touch on some off-label treatments in orthostatic hypotension i'll point those out as we go along there was a one extra slide and some errors that i fixed and i'll point those out as i go along so you can take some notes compared to the slides that you have on your flash drive but uh minor things okay so as you already heard the autonomic nervous system is an important system and i always tell patients this is the part of the nervous system you never have to think about until it stops working so it's an automatic system it's involuntary and it's really the role of the autonomic nervous system is to maintain homeostasis and we as uh as as humans are you know moving through different environments or up and down uh different levels of activity and so we this is an important um part of the nervous system for us it controls the involuntary functions as you heard about including uh including blood pressure heart rate digestion and other things and it's largely a motor system as you heard although there's afferent parts there's some sensory input in and largely the autonomic nervous system is a a reflex system it responds to need to go up okay uh thanks uh so there are some afferent inputs that are beneath our level of um perception that stimulate responses and and maintain homeostasis and as you heard there's three uh if you wanna simplify to three functional components of the autonomic nervous system sympathetic parasympathetic and an enteric system and i think it's useful particularly when we're talking too useful for us but also when we're talking to our patients to help them understand these three systems as uh in terms of what they do and it's not as simple as a push and pull system but this is a pretty good conceptual way of thinking about the parasympathetic nervous system as a rest and digest system it's the system that's mostly active as we're at rest conserving energy rebuilding our resources and then the sympathetic nervous system which is you know called the fight and flight response when we need to be physically active or in a stressful situation and different components of the parasympathetic and sympathetic nervous system are active all the time in relative amounts um and and you've already seen this uh in a different format but this is kind of the anatomy of the autonomic nervous system where you have sympathetic outflow from the thoracic and upper lumbar cord and the parasympathetic outflow from the cranial nerves and this and the sacral system as well so as content and of course as you heard the vagus nerve is the probably the major outflow of the parasympathetic nervous system so we talk about the uh problems with the vagus nerve or stimulation the vagus nerve we're talking about a major autonomic uh part of the system and then also just one other anatomical point um what's interesting about the autonomic nervous system is it's the one place uh in the body where you have uh neuronal synapses outside of the central nervous system so in the case of the sympathetic and parasympathetic nervous system there are these ganglia the sympathetic ganglia many of them lie in in a chain on either side of the spinal cord uh and then you have ganglia within the uh many ganglia many synapses within the enteric nervous system in the wall of the gut and so these are all nerve to nerve synapses that have their own autonomous uh crosstalk going on uh outside of the central nervous system in the parasympathetic nervous system the um the neurons in the in the brain stem or spinal cord have a long axon that goes out to the ganglia which sit in the right in the region of the of this of the end organs so in the case of the heart for example the parasite ganglia sit right in the pericardial fat the right close to the end organ and the sympathetic nervous system you have these gangly that sit closer to the spinal cord so these are short preganglionic neurons and longer postganglionic neurons and why does that matter because there there's some differences the these postganglionic axons are what we call c fibers they're small unmyonated fibers so would be affected by conditions that that cause small fiber neuropathy whereas the uh preganglionic axons are lightly mildly they're they're b fibers so less affected by uh things that that affect on my native fibers and then the other interesting part is in the synapse there's receptors these are acetylcholine receptors that are important for um uh transmission of all autonomic function through the parasympathetic or sympathetic or enteric nervous system so these are the nicotinic cholinergic receptors and at the end organs there's some differences because um the sympathetic nervous system largely uses norepinephrine as its neurotransmitter which of course interacts with alpha and beta receptors on the different end organs whereas the whereas the parasitic nervous system largely uses acetylcholine interact with muscarinic receptors and then you may remember that there's one exception to this rule and that's the sweat glands which are sympathetic are innervated by acetocholine from the sympathetic nerves so but for the most part sympathetic is adrenergic norepinephrine and parasympathetic is cholinergic we sometimes use those terms interchangeably we say someone has a cholinergic neuropathy we mean that's largely parasympathetic although also includes the sweat the sweat function and then the ganglia are our cholinergic synapses as well now we think about the autonomic nervous system it's really as i mentioned a reflex system so you have reflexes at every level of the of the autonomic nervous system so some you have some simple reflexes that occur right at the level of the blood vessels and skin there are some reflexes that occur at the level of the ganglia so they can occur without needing the uh needing the central nervous system these would be mostly like enteric reflexes uh that the the systems that produce peristalsis and other things their spinal cord reflexes so some of our basic reflexes for uh defecation and micturition have spinal reflexes but then a lot of this happens more centrally so reflexes that control blood pressure are mediated through the brain stem inputs from the bare receptors come to the brain stem and then a response to control blood pressure goes out from brain stem so it's not something we're aware of that's hard for us to know is our blood pressure up and down and what's going on with our sympathetic outflow it just happens and then you go a little bit higher up to some of the autonomic centers that dr santorini talked about including the hypothalamus that control more complex functions like circadian rhythms and temperature regulation and so on okay so we're talking about autonomic dysfunction one of the main reflexes we should be familiar with is the bare receptor reflex or barrel reflex this is the reflex that controls blood pressure and it's important for humans because we're you know one of the few animals that goes around with our brain five or six feet above the above the ground of course probably very important reflex for giraffes i imagine but in any case i learned from research on mice and rats that the bear reflex is not so important in four-legged rodents and that makes research a little bit difficult in this area so what happens when you when you go from laying down to standing up there's now a gravitational force that's tends to want to pull some of your blood volume down to your lower extremities and lower parts of your abdominal regions and that effectively would remove blood volume from the effective circulation if you did nothing else you would start to have decreased venous return to the heart and eventually drop in cardiac output and drop in blood pressure we don't want that to happen so as the uh there are some baroreceptors some afferents and some sensory organs up in your neck and and in the aortic arch which are stretch receptors they detect that there's been a drop in pressure and that that bare receptor input to the brain stem goes down so there's a reduction in the bare receptor output and that leads to a certain number of uh changes in the in the reflexes on both the parasympathetic and sympathetic side so simplistically you unload the bare receptors that's what it's called and as a result you have a reduction in the inhibition of the sympathetic nervous system and an increase in sympathetic outflow while at the same time you have a reduction in the stimulus of the parasympathetic nervous system which leads to a reduction in vagal outflow so the the end result is that you reduce vagal tone which allows the heart rate to rise and you increase sympathetic tone which stimulates the heart to increase could increase heart rate and cardiac output but most importantly increases the peripheral vascular resistance so that you how you squeeze some of that that blood so you have improved venous return in increased um peripheral vascular resistance and the heart rate goes up trying to force the blood to go the blood pressure to stay up in the blood to go to your brain that's the general gist of it and so it's a complicated system unfortunately we don't have to think about it very much until it doesn't work so this reflex this bare receptor reflex actually works in it's important because it works in both directions so we think about when we stand up and this this reflex preventing our blood pressure from dropping but on the other hand um when our blood pressure gets too high the bare receptors work in the opposite direction so if when the blood pressure drops this increases sympathetic outflow and supports blood pressure when the blood pressure rises the exact opposite happens there should be an increase in the vagal output to lower the heart rate and a decrease in sympathetic tone and these are important mechanisms to kind of keep our blood pressure in an appropriate homeostatic range not too high and not too low why do i bring that up because when we're talking about autonomic failure in just a minute you'll see that these patients have not only trouble with orthostatic hypotension which is what they'll complain about but they also can have pretty significant supine hypertension which can be a problem and and a lot of times they'll show up with the quote-unquote labial blood pressure if someone hasn't made the connection that the blood pressure is really just following the position it just appears to be all over the place that's what patients will tell you my blood pressure is all over the place okay so the end the end result is orthostatic hypotension and i'll just you know briefly tell you what the the definition is and also mention that dr sandrotti mentioned putting people in a room and keeping them there until they make some decisions so we had a consensus conference on uh management of orthostatic hypotension and that's just the results of that just been published in the journal of neurology that's a nice article if you want to take a look at it just i think came out in january of this year so anyway the definition of orthostatic hypotension is nothing new uh it's defined as a drop of systolic blood pressure of at least 20 millimeters of mercury and or a drop of dyestock pressure of at least 10 millimeters of mercury within three minutes of standing and the three minutes part is is kind of important because um lots of people many of us in this room perhaps if we are a little bit hypovolemic this morning if you stood up quickly out of bed you may have a brief lightheadedness which then goes away within 30 seconds or so that's just the amount of time it takes for your nervous system to kick in so we're that's a normal response we're not really going to call that orthostatic hypotension or if we do it's going to be transient orthostatic hypotension but people have autonomic failure will or failure of their bare receptor function or bare reflex we'll have persistent orthostatic hypotension we use three minutes as a you know general idea that so someone if you send your nurse in to measure orthostatic blood pressures and they come back in a minute and they give you the blood pressures you can tell them that they didn't stand up that patient long enough so that's important the other important thing about three minutes is that there are some patients who have milder forms of autonomic failure who develop orthostatic hypotension gradually over time and if you don't wait long enough you won't see it there's even some studies on delayed orthostatic hypotension which occurs after 10 minutes and those patients are at risk of all the similar sorts of morbidity mortality as people with oh but it's harder to pick those up on the standing test or even on tilt table test so that's orthostatic hypotension and there's lots of potential causes and really um autonomic disorders or neurogenic orthostatic hypotension uh is probably the minority of the patients who have static hypertension we see a lot of older patients who are on vasodilator medications who have symptoms of oh uh lots of people have hypovolemia for one reason or another uh who have static hypotension so we need to make sure that when we detect the symptoms and signs of of oh that we understand uh that we've eliminated some of the more common causes before we conclude that there's a neurogenic or the static hypotension and orthostatic hypotension is a is one of the prominent symptoms of autonomic failure which is the talk we're having right now so these are conditions where the autonomic nervous system doesn't work and in patients who have autonomic thera can be limited to the sympathetic or parasympathetic or can be a pan-autonomic dysfunction the symptoms of sympathetic failure would be predominantly orthostatic hypotension although anhydrosis loss of sweating is also a sympathetic function and one clue that this might be a neurogenic cause is when these patients stand up and their blood pressure falls they fail to increase their heart rate like you would expect if you're just hypovolemic and had a normal autonomic nervous system as your blood pressure dropped your heart rate should rise to try to compensate so that's a clue that this is autonomic failure as opposed to a another cause of orthostatic hypertension and on the parasympathetic side um patients will complain of decreased salvation dry eyes and dry mouth sexual dysfunction bladder dysfunction they generally don't complain that their pupils don't respond to light but they may complain of kind of difficulty focusing or glare and then the enteric nervous system can be very problematic so constipation and or diarrhea and nausea vomiting just general symptoms of generally hypomotility occurs so as patients come to us with autonomic symptoms there's a wide range of things they may complain about and many of these symptoms as you heard are from dr sandori are not necessarily specific for the autonomic nervous system someone complains of weakness or lightheadedness there could be other reasons constipation obviously and exercise and tolerance fatigue nausea and vomiting lots of different causes but in in the context of many of these symptoms occurring together we can start to worry about autonomic disorders or autonomic failure and when you do the examination at least what we do in neurology there's usually not a lot to find if it's a pure autonomic disorder their motor and sensory function usually is pretty normal so i like this approach so people tell you they feel dizzy when they stand up and what's the cause well you have to make sure they're not talking about vertigo you know positional spinning sensation which is not uncommon maybe they just have imbalance so a lot of our parkinson's patients say they feel dizzy what they really mean is their balance isn't very good and the ataxia patients will tell you the same thing and they don't really have any autonomic dysfunction or at least that's not what they're talking about we're really trying to tease out are they talking about lightheadedness they're talking about symptoms that sound like poor perfusion of their of their brain and then we have a few patients like one i saw this week uh who sent for autonomic failure who actually was demented and said her head felt sort of woozy and funny and she couldn't remember things so that's not really autonomic dysfunction either so we'll talk about the light-headedness group and you know you can break these out into a number of different categories as you heard from and i think we all kind of have the same formulation is this orthostatic hypotension we can check just lay them down stand them up check the blood pressure is this postural tachycardia or or some other dysautonomia where the there's no particular drop in blood pressure but perhaps an excessive rise in heart rate that leads to symptoms or is this an episodic syncope where many they can stand up most times and feel fine but occasionally under certain circumstances will drop their blood pressure suddenly and pass out so this uh this slide's not in your packet but i'm happy to share just a way of thinking about when you do a stand test or tilt table test what should you really see because you can tease out what category your patient falls into when they're complaining of orthostatic symptoms so when we when normal people stand up you're well hydrated you have a normal autonomic nervous system typically your blood pressure doesn't change and your heart rate either doesn't change or may go up a few a few points um that's normal that's your that's your bare reflex doing its job uh if you have orthostatic hypotension because you're low in blood volume not an autonomic disorder the blood pressure may fall and the heart rate goes up that's the normal response to that stress of the system that's also a normal autonomic nervous system you just need more volume or stop taking your diuretic in postural tachycardia we typically see either no change or a slight increase in heart rate excuse me blood pressure on standing but the main finding is that the heart rate goes up excessively um and there's you'll you'll hear about the guideline the the diagnostic criteria 30 beats per minute elevation with a normal blood pressure or 40 in in younger patients so that's defines pot so there's lots of different things then you need to think about why does this person have postural tachycardia and then people have syncope or vasovagal syncope or neurocardiogenic syncope oftentimes if you stand them up under or put them on a tilt table test you see nothing at all or a normal response but if they were to have an episode you would see a sudden drop in blood pressure and heart rate both that occur and some people try to tease out whether it's mostly the heart rate that drops or most of the blood pressure but in my experience they both kind of happen together in a quote unquote vasovagal episode okay so those are all different patterns we might see and and these are we sometimes might call it dysautonomia or dysfunctional autonomic nervous system but not autonomic failure in autonomic failure when someone stands up you you see a dramatic drop in blood pressure because they don't have the bare reflex working with really no significant change in in heart rate so really not difficult to distinguish neurogenic orthostatic hypotension from pots those are different things but i put this slide in here because uh lauren said that probably some people wouldn't understand would need some uh concept of that okay so there it is um and and this is a vocabulary um lesson so dysautonomia is really a generic term it means that the autonomic nervous system doesn't work right that's all it means but in in common practice we often use dysautonomia to mean that the autonomic nervous system doesn't work right although the autonomic reflexes are intact the wiring is all there just something's not working correctly and it may be either an excessive or inappropriate response to to stimuli whereas autonomic failure specifically means the autonomic nervous system either the nerves or the central pathways don't work and that's then there's failure of the system so there can be some overlap between those things so i wanted to um sort of make a few cases of autonomic failure just to uh kind of tie into what some of the disorders that paula mentioned earlier so here's a quick case so here's a 40 year old obese woman she's complaining of excessive sweating lightheadedness and burning discomfort in her feet she also has some nausea vomiting shortly after eating she gets some nerve conduction studies which are normal and on her exam she has reduced reflex a little bit of loss of sensation to pinprick and her feet are dry and she has elevated fasting glucose so what's the diagnosis there's a quiz okay so this is um the kind of thing you'd see in a diabetic small fiber neuropathy where you have both sensory and autonomic features and oftentimes patients with peripheral autonomic drop these will complain of excessive sweating which on the surface you say why are they sweating too much it could be compensatory because they don't sweat in their limbs they sweat a lot and if you ask them more more detailed questions they'll tell you they sweat in their head and upper chest predominantly and that can be disturbing to them so the feet are dry because the feet don't sweat and there's lots of pinprick sensations those small fiber nerves are affected so that's a small fiber neuropathy and if you were to test this patient you might see you'll hear about autonomic testing but that she has impaired sweat responses heart rate ver doesn't vary quite as much as it should during deep breathing uh and then if you tilt them up tilt her up you'll see a drop in blood pressure usually in these peripheral autonomic disorders it's not a not as dramatic as the more severe autonomic failure but but nevertheless meets the criteria with very little change in heart rate so this is a meets the sort of pattern of a neurogenic orthostatic hypotension in the context of an autonomic neuropathy okay so sometimes called a peripheral autonomic property and if you like to be cute you can call this a pan to go with the pots there you go okay so peripheral autonomic drop these are usually ones that affect the small fibers small fibers those c fibers you saw in the picture little tiny things you can barely see and these are the small fibers that carry autonomic and and pain information so when you do the nerve conduction study you shock the nerves you only only are really stimulating the large fibers and typically the nerve connective studies will be normal and then patients may have been told you don't have a neuropathy but you can evaluate these these with either autonomic testing or with skin biopsy this isn't a skin biopsy this is a nerve biopsy with electron microscopy just showing like you saw previously there are big myelinated nerves and small bundles of unmyelinated nerves live there so um a diabetic poly diabetic peripheral autonomic neuropathy about third it's estimated about 35 percent of diabetics will develop autonomic deficits at some point they often have gi dysfunction and they can have this compensatory hyperhidrosis but fortunately severe autonomic failure uh is uh which doesn't occur in most diabetics only about five percent but when it does occur it's pretty significant because there's there's good data that diabetics with severe autonomic failure have a fairly high risk of mortality and other complications interestingly predominantly cardiovascular complications i think this is really a the autonomic dysfunction's a biomarker of bad autonomic control okay so we should we all see that type type of case so here's case number two it's a 56 year old woman who has a more rapid onset over two weeks nausea and vomiting after eating severe constipation and dry mouth trouble with their urination vision gets dim on when she's standing up so it kind of sounds like she has some orthostatic symptoms and she was healthy except she's a smoker and we did her neuro exam it's normal normal strength reflexes sensation but her pupils kind of react slowly to light again nerve conduction sites and emg are normal and when she stands up she has a pretty dramatic drop in in blood pressure with absolutely no change in heart rate so again the pattern of autonomic failure this time on a sub-acute presentation which is sort of pan-autonomic because you've got the enteric and the bladder and other things going on seems like it's getting louder so uh paula touched on this this is sort of a subacute autonomic disorder and you think about either a perineoplastic or autoimmune autonomic ganglionopathy in this particular case you got pupil involvement which you don't really see in peripheral autonomic neuropathies and as you heard the autonomic autoimmune autonomic ganglionopathy is um is not common but we see um you know in autonomic centers we'll see a few cases new cases every year subacute onset severe orthostatic hypotension gi symptoms very prominent but also urinary symptoms in more severe cases you can actually see the pupillary involvement many of the idiopathic cases have ganglionic acidocone receptor antibodies and the perineoplastic ones may have a different antibody profile depending on what cancer they have and these are patients that are important to identify because they're potentially treatable as you heard some of them get better on their own but my experience is they don't get a lot better they still they can go go about life but are still not very happy but they will get better with a plasma exchange and i like to present this rare disease to cme groups because you have to at least be aware of it i saw a patient just last week who had been undiagnosed for 13 years before we figured out this is what she has and she hopefully will get better with treatment and i just throw this in there too so this is a type of autonomic failure and pots is not autoimmune autonomic ganglionopathy um there's really no connection between those two except that in some previous studies some large groups of pots patients there were some uh small percent of patients who had this ganglionic acid cold receptor anybody usually had very low titers and uh without the features of aeg so uh just because someone with the pots phenotype has um low titers of ganglionic acid code receptor antibodies which at low titers are not all that specific uh i don't think you need to make the diagnosis of aeg okay that's the teaching point there okay third case um seven-year-old woman with parkinson disease who was on levodopa doing pretty well but then started getting complaints of lightheadness and occasionally falling off and after meals and her supine blood pressure was a was high 180 over 84. uh when she stood up her blood pressure dropped by over 100 points and she felt a little bit bad when that happened and when we tested her she had predominantly adrenergic autonomic failure with the orthostatic hypotension and her diagnosis was of course parkinson's disease with autonomic failure because uh parkinson's disease is an alpha-synucleinopathy a neurodegenerative disease and alpha-synuclein likes to get into the autonomic nerves and cause mischief as well this can be sort of classically you know i learned in medical school that these non-motor manifestations of parkinson's disease were late manifestations but i think uh those of us who have autonomic practices realize that that's often not the case that autonomic dysfunction is there early on in the disease and maybe later it gets more severe and it can even be quote-unquote the presenting feature some patients present with purely autonomic dysfunction orthostatic hypotension predominantly we'll initially call them pure autonomic failure but then over the next few years they develop parkinsonism and we know that they're either developing multiple system atrophy or or features of idiopathic parkinson's disease so parkinson's disease often affects more the adrenergic the dopamine and norepinephrine neurons more than the cholinergic ones and and symptomatic orthotic hypertension in some estimates uh neurogenic orthotic hypertension is found in about 18 of parkinson's disease patients at some point um but again if it's if it's occurring early and it's more severe particulars bladder involvement we should be thinking about maybe this parkinson disease is turning into msa and not idiopathic pd um and then one last case uh just to share a different presentation so here's a 50 year old man who had hodgkin's lymphoma so he had his neck dissected lymph nodes taken out radiation and chemo to his neck 10 years ago so he's doing great hodgkin's disease all taken care of but now he comes in he's complaining of label blood pressure high blood pressures and low blood pressures without any kind of pattern to them and in fact when we put him on the tilt table his supine blood pressure is ridiculously high and the standing blood pressure is quite low and his heart rate actually goes down when he stands up it's just all messed up so um so he has baroreflex failure and in this case it's a bare receptor failure it's an afferent problem and probably all of his efferent nerves that go out to the blood vessels are just fine his but his brain doesn't know what his blood pressure is so he can't control it so this is not uncommon but yeah obviously the context will tell you the answer someone's had their neck messed with and interestingly you can make this diagnosis because even though their blood pressure doesn't respond appropriately to posture if you stick their hand in cold water you'll get a you get a pressure response um it tells you that the autonomic sympathetic nerves are still intact so this is bare receptor failure due to crowded sinus damage okay so autonomic failure comes in different forms and the these two categories are the ones you're most likely to see in practice so there are the neurodegenerative diseases which for the most part are living in the family of alphas and nucleanopathies so we don't see significant autonomic failure in alzheimer's disease or other amyloid or talopathies and if someone comes in with parkinsonism and falling all the time and they don't have vertical eye movements they have psp or talopathy and they don't have they typically don't have orthostatic hypotension and these alpha's nucleophiles affect both the central and peripheral autonomic autonomic networks and there's a peripheral autonomic drop these and ganglionopathies that you already heard about a little bit diabetes and amyloid being common ones but autoimmune conditions are not uncommon and then inherited autonomic drop these uh are probably more common if you're a child neurologist than as adults but they do occur and then there's a special cases uh the spinal cord injuries and bear receptor failure which which occur as well and there's your pans we'll hear about pots later okay so neurogenic orthostatic hypotension i hopefully have made the point is is orthostatic hypotension due to autonomic dysfunction not not due to drugs or hypovolemia and uh it's orthostatic hypertension often without an appropriate compensatory tachycardia that's a clue of course we do have patients who have pacemakers and other sorts of things as they get older which limits their uh heart rate response and they have to kind of take that into account so these patients can be lightheaded dizzy and faint when they stand up but important to remember that those aren't the only symptoms other patients with noh have uh they will profess up and down they have no dizziness a lot of my parkinson's patients i'm not dizzy but i have this pain in my neck every time i stand up or i just and or the wife says he just doesn't seem right when he's standing or my legs feel like they're buckling so uh a more generic question like do you have any symptoms that are worse when you stand up that get better when you sit down might be a good screening question as opposed to fixating on lightheadedness per se they may have postprandial hypotension that's a clue they may have supine hypertension so if you lay them down their blood pressure is too high which would again suggest maybe they have neurogenic form of static hypotension and some of the patients with alphanucleopathies and degenerative disorders have a phenomenon called reverse dipping so most of us if we have a normal circadian pattern of blood pressure our blood pressure goes down at night and it comes up in the morning as we're getting ready to go about our day and unfortunately people with these some of these autonomic disorders get reverse dipping but their blood pressure gets real high at night and then it drops in the morning and so many of the patients will say their orthostatic symptoms are worse first thing in the morning they're just getting up for the first time they're a little bit hypovolemic and their blood pressure circadian blood pressure is dropping right then so if you get a story that my blood pressure symptoms are worse in the morning but get better over the rest of the day again maybe a neurogenic cause could be thought about and that's because the symptoms in chronic conditions may get less even though the the actual disease may be getting worse okay so i'll spend the last little bit of time talking about our management of neurogenic orthostatic hypotension and the most important thing here is that our goal is to help relieve symptoms uh and not to normalize blood pressure because you can imagine there's no way we're going to normalize blood pressure it's too high in some scenarios and too low in others and in fact being aware of this condition is important because some patients may come into their primary care doctor's office and they sit them in a chair and measure their blood pressure and go it looks great your blood pressure is just fine not realizing that if they were lay down it'd be ridiculously high if they were to stand up it's going to be symptomatically low so we have to actually measure the right blood pressure but not to fixate on blood pressure and to educate our patients not to fix it on blood pressure too much because we're trying to improve their quality of life and relieve their symptoms without putting them at risk of bad complications so number one if there's an underlying cause we try to address that and some people with neurodegenerative autonomic failure also have also are taking a diuretic or a vasodilator and also hypovolemic so we can fix those things and that's often very helpful important to review their medications as i said so innocently their urologist may put them on an alpha blocker and try to get them peeing better but often makes their blood pressure worse so look at those things increase the fluid intake so the one error in the in the notes is that i got the volume i got the um the volume wrong so one of these little bottles from the grocery store is usually about 16 ounces which corresponds to 500 milliliters so if i if i had done the math right that would have been four bottles equals two liters so this is correct um but you could have figured that out on your own so we want them drinking at least two liters a day there was some guidance that came out forget where it was published that suggested maybe for normal humans three liters is even better but at least two liters is what i recommend my patients you'll be surprised how many older patients particularly older men who self-restrict their fluid intake because they don't want to get up at night to go to the bathroom and they drink a very you know sometimes extreme exceedingly small amount in the course of a day salt is a little more controversial in young healthy hearts you can increase the salt intake and that helps to increase volume one teaspoon of salt is 2.3 grams of sodium so two or three tablespoons of salt but i always use caution so if someone has a past history of cardiac disease or or long-standing hypertension then i might be a little less push the salt a little bit less so you have to individualize that oops what happened and then recognize and avoid activities that can worsen orthostatic hypotension if you ask patients they'll tell you that they feel worse standing up they feel worse in the morning they feel worse when it's hot and then they say doctor why do i feel so bad when i'm taking a hot shower in the morning you have to say well that's because you just told me why you do that so hot showers in the morning probably not um so a little change in your lifestyle can make a big difference avoiding large meals and alcohol so other patients say i go out to dinner have a nice big meal drink a little wine and then you know i have to be carried out of the restaurant and that's embarrassing so as long as they're kind of aware that they need to be cautious about what they're doing elevating the head of the bed can be very useful i think i have a slide coming up to show that using physical counter maneuvers when the lightheadedness comes on to try to avoid syncope and give yourself a little time to find a place to sit down a little bit of recumbent exercise to keep the muscle tone up recumbent because i tell my patients who have significant static hypertension the treadmill is a dangerous place it's dangerous anyway but um uh recumbent exercise and then compression garments can be quite helpful so uh just going through a few of these uh elevating the head of the bed is interesting actually actually roger bannister uh who you may remember is the man who first ran a four minute mile published this back in the 60s that if someone slept with their head elevated on an angle like this that their degree of mourning orthostatic hypotension this is the drop from supine to standing was um was less just from sleeping with their head up um compared to earth stack hypertension as well while sleeping in the flat position and but still patients are resistant they don't want to mess with putting blocks under the head of their bed and so on until you tell particularly the men that you'll pee less at night and they're like well that's great i will do it right away and then a lot of times i find this quite helpful even though it seems like a minor intervention particularly when you consider that most people can only get the head of their bed up by eight or ten inches if you try to go higher particularly if you have the 600 count silk sheets you know you're going right out so and they don't have to get a hospital bed or anything expensive this this little intervention can be quite helpful and i also tell my patients you have supine hypertension so really your days of laying flat should be over and done with anyway so you should never be laying flat and when you do lie down it should always be with your head up like this physical counter maneuvers help a little bit um you can briefly get your blood pressure up by 10 millimeters or so if you uh squeeze your lower extremity muscles that sort of pump some of the venous return back to the heart and the best way is sort of buttock clenching it's hard to demonstrate to patients in the office but it does work patients can squat bend over grip their hands and some will learn if this is helpful for them to do a few of these things just before they stand up to give themselves a little boost this is interesting and most people don't appreciate this as much but there is a um a osmo presser reflex that that is present in humans so if you drink a lot of pure tap water nothing in it you'll briefly lower the osmolality in the portal circulation and that leads to a pressure response and in normal people who have normal autonomic nervous system you don't see it because the barrel reflex takes care of it all evens out but in people of autonomic failure you'll see this osmo pressure reflex as an increase in in blood pressure that occurs within a few minutes of drinking that bolus of water and it can last for up to an hour so it's a pretty impressive reflex and and the increase in blood pressure can be meaningful in some patients who are just on the border of feeling light-headed or passing out so i tell patients if they can to drink four or five hundred milliliters of water it's one bottle first thing in the morning and you have to drink it down fairly fast i don't know what you tell patients but as fast as you can and then then you should be able to get up and go a little bit better and again some patients find this very a very pronounced effect and they use it to their advantage and others don't notice it as much but it's it's another easy mechanism that doesn't require medications and can help alleviate especially morning orthostatic hypertension but at some point you do all those interventions you got the abdominal binders and the spanx and the and the water and uh salt and you got the head of the bed up patients are still symptomatic there are medications that really fall into two main categories the first being the sympathomimetic medications so these are ones that increase blood pressure mitogen is an alpha alpha 1 agonist so it just causes peripheral vasodi vasoconstriction raises the blood pressure both laying down and standing blood pressure so with any of these sympathetic medications patients must understand that if they're taking a tid that means morning noon and afternoon not bedtime because you're not supposed to lay down for at least four hours after you take one of these medicines okay because the spine hypertension which is already there is going to be worse so um 2.5 to 10 milligrams tid are typical doses side effects can include not only hypertension but also the other interesting symptom is scalp itching or prickly feelings because you're activating these sort of scalp receptors as well okay so that's available as is droxy dopa or north era this is a norepinephrine precursor drug that's recently approved by the fda recent you know about a year ago so this works a little bit differently it increases norepinephrine levels which may be how it works but it also increases blood pressure a bit and improves improved symptoms so again no laying down within four hours of taking one of these medicines okay all the other drugs besides these two are off-label and that includes things like ephedrine or godamines and then the what i call the antidiuretics so drugs that make you retain volume retain salt so the main one people use is plutocortisone which is indicated only for adrenal failure but you know who cares we use it for other things so this is a mineralocorticoid that causes sodium retention but may cause hypokalemia as a side effect and typically 0.1 to 0.2 milligrams per day at least in my experience using more than that just leads to more side effects and and less and not more benefit some people use desmopress in particular they have a lot of nocturnal diuresis this is also an antidiuretic type of medication and then there's a whole bunch of other things that people try um they're listed here including pyrostigmine which is thought to help to improve ganglionic transmission and cholinergic transmission and that can be helpful and then some centrally acting agents but these are all sort of used by more in more severe cases by people with a lot of experience and then remember that anything that supine hypertension is also a problem and don't forget about it then they can have severe supine hypertension before you've even started your intervention so elevating the head of the bed no pressure medications toward the end of the day water intake in the morning but but less so as the day goes by and then dr kaufman likes to give people the option of taking some candy and wine at bed which is nice and then occasionally someone who's got really significant spine hypertension that's limiting your ability to treat their other symptoms you may need to add a short acting anti-operative at bedtime it's not that complicated so i like to i favor the ace inhibitors and arbs some people like the nitro paste something that's going to be mostly worn off or can be taken off in the morning so that's uh that's orthostatic hypotension and autonomic failure uh just in summary autonomic disorders are common but dysautonomia and autonomic failure don't mean the same thing so careful history examination review of the medications that's critical and really anybody can diagnose orthostatic hypotension it's not that challenging but sometimes in a complex case particularly you know some of those neurodegenerative ones you need a more uh expert assessment with autonomic testing and realize that in some cases uh uh orthostatic hypotension autonomic failure is what it is and other times it's a sign of a impending neurodegenerative disease that's coming on and the symptoms are treatable uh even though many times the cause is not is not fixable very good so i'll stop there and i guess questions are later you

Channel: Dysautonomia International

Views: 1,529

Rating: 4.8857141 out of 5

Keywords: autonomic, neurology, doctor, medical, hypotension, dysautonomia, neuroscience

Id: q_nPiwXswos

Length: 44min 37sec (2677 seconds)

Published: Fri Jul 24 2020