CurePSP and the Mayo Clinic co-hosted a conference in late June 2018
on progressive supranuclear palsy, corticobasal degeneration, and
multiple system atrophy. Here’s a link to the video recordings of
CurePSP and the Mayo Clinic co-hosted a conference in late June 2018
on progressive supranuclear palsy, corticobasal degeneration, and
multiple system atrophy. Here’s a link to the video recordings of
This is a story of a family coping with multiple system atrophy (MSA):
Update on 6/11/18: Unfortunately this webinar is now cancelled.
Brain Support Network group member Steven Russell shared with me today info on an upcoming webinar, which will give an overview of all four of the atypical parkinsonism disorders — MSA, LBD, PSP, and CBD. Details are:
What If It’s NOT Parkinson’s?
In this webinar, Dr. Mihaela Alexander, Colorado Neurodiagnostics, will discuss atypical parkinsonisms such as multiple system atrophy and Lewy body dementia, among others (PSP, CBD). Dr. Alexander will cover what we know about these conditions and what treatments are available for each condition.
Tuesday, June 12
10-11am CA time
Parkinson Association of the Rockies, Denver, parkinsonrockies.org
General Information: (same as what is above)
The International Multiple System Atrophy conference was held in early
March 2018 in New York City. In late April, an article describing the
highlights of the conference was published by NYU’s Dysautonomia
Center. Check it out….
On Tuesday, Jackie Vandenberg, who lives in NY with multiple system atrophy, asked people to share her open letter to family and friends. The goal of her letter is to help family and friends communicate with her better. I think it’s worth sharing with everyone as it is not MSA-specific but person-specific. Others may want to use this open letter as a model for their own letter to aide in communicating with your family and friends.
Dear friends and family,
I always appreciate when friends and family come to visit. I noticed
lately that some people struggle knowing how to talk with me as my
ability to communicate decreases. I have brainstormed a few things to help…
1) I enjoy socializing even though I cannot speak back. I’m interested
and am listening when others tell me about themselves. I like to hear
about how people are doing, their families, hobbies, vacations,
experiences, weekend activities, etc.
2) I wish to be spoken to as a person and an adult. Please speak to me
normally by not using slower or louder speech, or a reduced vocabulary
and sentence structures. I like to be part of the conversation by making
eye contact and being spoken to directly.
3) It’s easier/quicker for me to be asked ‘yes’ or ‘no’ questions.
4) When we’re together you can talk or not talk, either is fine. I’m
comfortable with quiet too.
5) I use a piece of cardboard with the alphabet written out to
communicate, we call it a Ouija Board. I can point to letters to spell
out words. I need someone else to hold the cardboard and adjust it as I
go. It can take a while for me to spell out sentences so I appreciate
everyone’s patience. It’s ok if you don’t understand a word the first
few times, as Tom and Adrianne can attest to.
6) When I’m using the Dynavox communication device it’s ok to fix
letters/autocorrect for me.
7) A symptom of MSA is excessive drooling. If you’re comfortable doing
so you can use a tissue or washcloth to wipe my mouth.
8) The best way to contact me to make plans is on Tom’s cell phone
through call or text. I check my email and Facebook messenger every
couple of days and am happy to get messages there too.
I hope this list is helpful for everyone! I love having friends and
family visit and appreciate all of our time together.
Northern California-based Family Caregiver Alliance (caregiver.org) is
organizing a free educational program called SHARE.
The care receiver (person with a neurological diagnosis) must:
– Be over age 60
– Be living at home in the San Francisco Bay Area
– Have intact cognitive abilities – NO dementia
– Not be in the terminal stage of their disease
The caregiver will receive:
– 6 in-person home visits
– Learn effective communication skills
– Learn to reduce stress
– Learn to promote health
– Learn about long-term care management (make the most out of today
while planning for tomorrow)
If this is of interest, contact Michelle Venegas (415-434-3388, x323,
As 2017 ticks down, we hope you enjoy some quality-time with family and friends. We wanted to share our results for 2017. Plus, this is a great time to make a charitable contribution as other generous donors are doubling your contribution.
Brain Support Network (BSN) continues to pursue its three missions:
(1) create and disseminate information on LBD, PSP, MSA, and CBD to members (You are one of 450 Northern Californians on our network’s email list.)
(2) coordinate the local caregiver support group in San Mateo
(3) help any family with brain donation.
We (BSN volunteers and part-time employees) have kept busy in 2017:
Brain Support Network (BSN) continues to pursue its three missions:
(1) create and disseminate information on LBD, PSP, MSA, and CBD to members
(2) help any family with brain donation
(3) coordinate the local caregiver support group in Northern California
We (BSN volunteers and part-time employees) have kept busy in 2017:
* We sent out over 250 email updates, most of which focused on one of four specific disorders: LBD (Lewy body dementia), PSP (progressive supranuclear palsy), MSA (multiple system atrophy), and CBD (corticobasal degeneration). Many emails relate to caregiving and dementia caregiving.
* We published over 600 Facebook posts on similar subjects (because some people prefer Facebook).
* We served as a clearinghouse of information and support for network members.
* We have kept our web site relevant and up to date (e.g. our “Top Resources” lists for the four primary disorders and our blog).
* We organized 94 brain donations (a new record for BSN), most of which were delivered to the Mayo Clinic in Jacksonville. (Of course the year isn’t quite finished.)
* We hosted our largest-ever “Research Update and Practical Conference on PSP/CBD” in cooperation with the UCSF Memory and Aging Center on October 28, 2017, in San Mateo. Conference video and handouts are available.
This is the time of year when we ask you for assistance.
Five long-time support group members and two long-time BSN benefactors have offered a “challenge grant” to network members. Your charitable contributions through December 31st will be matched up to $8,000. Please help us take advantage of this opportunity and help make possible our efforts for the coming year.
If you mail a check, please write “match” on the memo line along with the name of the family member or friend that you are honoring or remembering. Or, enclose a note with that information. Make checks payable to “Brain Support Network,” and mail to BSN, PO Box 7264, Menlo Park, CA 94026. To count towards the challenge donation, checks should be dated and postmarked by December 31, 2017, Your check does *not* need to be received by this date.
If you make an online contribution (via credit card), please write the name of your family member/friend after selecting “in honor of” or “in memory of.” Please append “/Match” to the name of the person. To count towards the challenge donation, online contributions should be completed by Sunday, December 31, at 11:59pm California time.
Brain Support Network is recognized by the IRS as a 501(c)(3) tax-exempt charity and your donations are deductible to the extent allowed by law. Please know that any amount—$25, $50, $100, $250, $500, or more—is appreciated! Thank you for supporting our three missions!
Happy 2018 to you and your families!
Brain Support Network CEO
This is a good summary article from Neurology Advisor (neurologyadvisor.com) in mid-November 2017 about cognitive impairment in multiple system atrophy (MSA). Here’s a link to the full article:
This was the most interesting excerpt to me:
A 2014 position paper published in Movement Disorders by the MODIMSA Study Group observed that the shorter survival associated with MSA may mask a potentially higher rate of cognitive decline, as the incidence of CI in patients who live longer than 8 years is nearly 50%. “If the disease did not have such a rapid course, the cumulative prevalence of dementia in MSA would be similar to that of Parkinson disease,” they wrote.
The full article is worth reading!
This webinar from the Michael J. Fox Foundation from 2014 gives a very broad overview of several movement disorders *besides* Parkinson’s Disease (PD). Much of the webinar focuses on Lewy body dementia (LBD) though there is some discussion of multiple system atrophy (MSA) and a bit of discussion of progressive supranuclear palsy (PSP) — as these three diseases are often confused for each other and for Parkinson’s. And the webinar includes a terrific discussion with two physicians about research into these disorders.
What do Movement Disorders Look Like?
* Lou Gehrig’s Disease (ALS) – Gradual loss of muscle control, muscles atrophy
* Dystonia – Muscle spasms and contractions; repetitive, twisting movements
* Essential Tremor – Rhythmic shaking, most often in hands; most common movement disorder
* Lewy Body Dementia (LBD) – Cognitive impairment; hallucinations; spontaneous parkinsonism
* Multiple Sclerosis (MS) – Vision difficulties; balance problems; numbness and muscle weakness; thinking and memory problems
* Multiple System Atrophy (MSA) – Parkinson’s-like motor symptoms; more severe autonomic dysfunction
* Progressive Supranuclear Palsy (PSP) – Gait and balance problems; inability to focus eyes; cognitive impairment
Here’s a link to the recording:
Across the Spectrum: Parkinson’s and other Movement Disorders
Michael J. Fox Foundation Webinar
March 20, 2014
Brain Support Network uber-volunteer Denise Dagan recently listened to the recording and shared notes below.
March 20, 2014
LEWY BODY DEMENTIA
Alexander explained that he went 20 years before getting an accurate diagnosis of LBD. One symptom was significant fatigue, misdiagnosed as Chronic Fatigue Syndrome. Another was losing his sense of smell. 10-15 years into these strange symptoms was REM Sleep Behavior Disorder, which has since been closely linked to LBD, but at the time was not. Now, these things are considered early warning signs of PD, but at the time doctors didn’t suspect because he still doesn’t have any significant motor or gait symptoms. He was misdiagnosed with Alzheimer’s even in the presence of hallucination and perceptual symptoms. Only when he did his own research was he convinced he did not have Alzheimer’s, but LBD. He discovered a neurologist as a forerunner in the field of LBD and flew to him to confirm that diagnosis. At the time he was surprised at the ignorance of neurologists about LBD. He has since found they are most curious to learn about it.
Alexander is working on a humorous monologue called “Braking for Alligators.” He hallucinated, and braked for, an alligator in Massachusetts. He believes humor is very powerful in taking some of the weight off the experience of having such a serious diagnosis with disturbing symptoms, like hallucinations. Humor is something he can still offer others.
Dr. David Standaert is not surprised that 20 years ago doctors didn’t use the term LBD. The name was coined in the late 1980s and even in the early 90s they knew very little about it. It would have been called atypical Alzheimer’s or atypical dementia until the late 90s when they were able to find Lewy bodies in the brain and understand their significance in this neurodenegerative disorder.
Lewy bodies are an abnormal structure found in the dopaminergic neurons in Parkinson’s disease. In the late 90s, researchers discovered the protein alpha synuclein, which is a major component of Lewy bodies. That opened the door in looking across the brain. Researchers discovered that those people who had dementia and other associated symptoms Alexander described (including hallucinations) had these Lewy bodies all over the brain. These Lewy bodies are hard to see unless you stain for alpha synuclein, then they are obvious. LBD doesn’t typically have forgetfulness, like Alzheimer’s.
Dr. Susan Bressman says the abnormally mis-folded, or clumping proteins are a common phenomenon of other neurodegenerative disorders (MSA, PSP), as well.
Dr. Standaert believes that they will ultimately find that Parkinson’s Disease (PD) and LBD are the same condition (the basic disease process is the same in these two disorders) manifesting in different ways. Dr. Bressman agrees.
Alexander has participated in research at the Mayo Clinic. The DAT scan shows the dopamine deficit even though he doesn’t have typical movement symptoms. Dr. Bressman suggests the area of the brain affected determines what symptoms manifest so Alexander has RBD, loss of smell, hallucinations (pre-motor features). Alexander does take some Neupro, which, at a higher dose, worsened his hallucinations. He still takes a low dose.
MULTIPLE SYSTEM ATROPHY
Dr. Bressman says MSA can be clinically difficult to distinguish from PD. One form has a cerebellar effect with more unsteadiness and uncoordination symptoms. There is also a Parkinson’s form with really does mimic Parkinson’s. What helps distinguish it from PD are problems with autonomic issues like bladder and blood pressure control very early in the progression of the disease. It can take years to feel confident which diagnosis is correct. There is a lot of overlap in the pathology, but in MSA, instead of the neurons, alpha synuclein pathology is in the glia supporting cell. The glia cells in the brain have inclusions. Treatment overlaps as well.
Dr. Standaert agrees with Dr. Bressman. There’s no test to distinguish between MSA and PD during life. People are working on one. As a neurologist follows a patient over years symptoms become more distinct, like when motor symptoms do not respond well to PD medications, and when there are a lot of early autonomic symptoms. In MSA there are very few cognitive problems. Under a microscope, you would not mistake MSA for PD. It is still alpha synuclein, but it is in the glia in MSA rather than in the neurons in PD.
There is some loss of dopamine function in MSA because the Parkinsonian form does damage the substantial nigra, but the appearance on the DAT scan is somewhat different because in MSA you can see the damage is still somewhat even, whereas in PD the damage is asymmetric. So, the DAT scan can give you a clue, but it is not a definitive test to separate the two.
Dr. Bressman says there are papers suggesting an MRI can help to distinguish between the two, but there is a lot of debate about that. Doctors will sometimes send patients for a glucose PET scan to use the glucose metabolic pattern to distinguish between typical Parkinson’s and more of an atypical parkinsonism of some sort. The definitive diagnostic method is really to follow patients over time and watch the manifestation of symptoms, responsiveness to medications, and putting all the pieces together.
QUESTION AND ANSWER
Dave Iverson asked the doctors what can be learned about one of these neurodegenerative disorders as we learn about another of them. Dr. Standaert says they are all age-associated diseases. While young people do, occasionally, develop neurodegenerative diseases they develop after age 50, 60, 70 and beyond so age is a trigger. They are all also associated with the development of abnormal proteins. Each disorder is a different protein (misfolding protein), but at the core there are important commonalities.
Dave Iverson asked if there is an important reason to pursue the right diagnosis. Dr. Bressman says patients really want to know what it is. Knowledge is power, and getting the right diagnosis can affect getting the right treatment. When you get to MSA, PSP, CBD at this point the treatment are empiric for the most part. It is important in terms of prognosis, family counseling, clinical trials, and ultimately for targeted treatments, when those become available. We think of PD as being a homogenous entity, but there are subtypes, early onset, those with more or less gait disorder. So, on the one hand we lump them together, and on the other hand we want to customize treatment to each individual’s greatest difficulties.
Dave Iverson asks if essential tremor can progress to PD. Dr. Standaert says sometimes doctors will diagnose essential tremor (often symmetrical, runs in families, and is bilateral, so not PD) and the patient will return with real PD symptoms. People with essential tremor tend to be diagnosed with PD more frequently with PD than the general population. They thought this was due to misdiagnosis as essential tremor when it is incipient PD. DAT scan can help with this teasing out between these two conditions. Dr. Bressman totally agrees. This lingering question of whether essential tremor increases risk of developing PD, or is essential so common some percentage will go on to develop PD in the same numbers of the general population, or are some number of those diagnosed with PD misdiagnosed until the PD symptoms become obvious. That’s why we have the DAT scan. That’s what it is FDA approved for, to distinguish between these conditions. Dr. Standaert says if there is a mechanistic or genetic connection between essential tremor and PD, they haven’t discovered it, yet.
Dave Iverson asked if it is unusual for someone to have PD and then ALS, for example. Dr. Bressman says it is an unlikely but now that we have different genetic subtypes, looking at ALS through a genetics lens, it is a heterogeneous disorder and some people who have motor-neuron disorder can have parkinsonism or a PSP-like picture. So, the motor neuron picture is getting more complicated as we’re understanding the genetics. She has had patients with motor-neuron disease and parkinsonism who have turned out to have one of these genetic subtypes. It’s rare. They are separate disorders but in some subtypes you can have the two together.
Dave Iverson asked if the LRRK2 mutation that causes the most common genetic form of PD can also lead to other movement disorders. Dr. Standaert says in families where the original LRRK2 gene was discovered as a cause of PD (2-4% of cases in the US) some individuals had MSA or PSP (tau) -looking pathology. So there were other forms of neurodegenerative disease in those families. This indicates LRRK2 can not only trigger PD, but other forms of neurodegenerative diseases. Researchers wonder about LRRK2 — does something happen far upstream, modulating the response of the brain to these mis-folded proteins, perhaps modulating the inflammatory response that follows them. So, is it a general kind of gene that can enable a number of different pathologies?
Dr. Bressman has been looking for gene carriers that have these other neurodegenerative disorders or other phenotypes, but hasn’t found that so far. Family members who are gene carriers are either normal (healthy) or have PD, although it is classic PD. There is more of a gait/balance issue than a tremor. Some have a classic rest tremor. They haven’t identified motor-neuron disease or PSP or other neurologic pictures in these families. Only 28-35% of people who have this gene will develop PD before age 80. This seems to lead to a connection between a link between the gene and some upstream event, or some sort of exposure to lead to PD.
Dr. Standaert says most disease process are a combination between genetics and environment. We just don’t understand this enough in PD.
Dave Iverson asked Alexander if he has autonomic symptoms (bladder, constipation, blood pressure, etc.). Alexander says yes, he didn’t realize that they were associated to his illness until the doctor confirming LBD started asking him about some autonomic issues, specifically. Then he knew all his symptoms were related.
Dave Iverson asked Dr. Bressman if these autonomic symptoms cut across all these disorders? She says certainly PD and MSA and can be the most debilitating feature (like low blood pressure, and bladder issues).
Alexander comments (and the doctors both agree) that proper diagnosis is important, especially for those with LBD, because word needs to get out to doctors, patients and families to prevent patients being given neuroleptics (such as Haldol) which are powerful blockers of the dopamine receptors in the brain. These types of drugs are used widely in medicine when someone has hallucinations (common in LBD). If you give this to someone with LBD, even though they may not have symptoms that manifest as parkinsonian/movement related, they can become rigid and stiff for weeks.
Dave Iverson asked what is the difference in prognosis between these different disorders. Alexander says his doctor says, in his experience, the rate at which the initial condition unfolds is similar to the rate at which it further progresses. If symptoms come on gradually, it is likely to continue to progress just as slowly and is unlikely to make sharp downturns. That is good news for him as his took so long to diagnose.
Dr. Standaert agrees, although no two cases are exactly the same. The pace of one’s disease progression doesn’t change a lot over time. These neurodegenerative disorders progress at different rates from each other, ALS tends to progress much more rapidly than others.
Dr. Bressman agrees. There is no crystal ball because something new can happen as one ages.
Dave Iverson asked Dr. Bressman to talk about dystonia. She says dystonia is on the list separately because a not insignificant percentage of PD, particularly with early onset, can be caused by the disease itself or medication induced. How you treat it depends on what you think is the cause (peak dose, end of dose, early morning) so you may adjust the timing, Amantadine, or Entacapone. Ultimately, the best treatment will be better dopaminergic meds, DBS or a cure.
Dave Iverson asked if exercise helps in all of these disorders as it does for PD. Dr. Standaert thinks exercise is helpful in all of them, but in PSP there is a tremendous issue with balance and falling. MS is worsened by overheating, so be careful with that. Apply the right kind of exercise for safety to each disorder. Alexander says he only recently realized exercise is helpful for him.
Dave Iverson asked if there is a connection in both MS and ALS. Dr. Standaert says both have abnormal proteins, but the part of the brain attacked is different. MS is quite different as it is an immune attack upon the brain, but the commonality is the recent recognition of the inflammation response between all these disorders. Otherwise, the cause, diagnosis, and management is quite different.
Dave Iverson asks about the more drastic drop in blood pressure between in MSA than in PD. Dr. Bressman says that is true. The treatments are very similar, but too many patients don’t talk about it. If they are feeling faint they should tell their doctor and have regular blood pressure checks to discuss how to manage it. Its dangerous because it can lead to falling, but there are a lot of treatment options. Some people are even still on old blood pressure meds to lower blood pressure from cardiologists prior to adding a neurodegenerative disorder, and those aren’t needed anymore.
Dave Iverson asks Dr. Bressman if she is hopeful that connections between research will lead to treatments across all these disorders. She is quite hopeful and the research is broad and applicable to not only insight into PD, but other disorders with respect to the search for a cure or better uses of the treatments they already have.
Dave Iverson asks Dr. Standaert if he things that is encouraging for pharmaceutical companies. He says the more they learn about these diseases the more they realized there are shared commonalities of attack to research treatments. Success in one will really open the door to success in others. The rare disorders may not get the funding for research, but will benefit from those getting funding. PD may not be just one condition because there are more than one gene that can trigger it, and a multitude of symptoms. Dr. Bressman says one type of ALS may share a treatment option with some type of PD.
Dave Iverson asks Alexander to close the conversation. Alexander says he has found with respect to his hallucinations is to use them as creative prompts for writing poetry and other creative works. That is always potentially possible and there is more attention to this in the dementia care community.
Brain Support Network and Stanford University co-hosted a webinar last Monday, September 18th about orthostatic hypotension (OH) in Parkinson’s Disease (PD), Multiple System Atrophy (MSA), and Lewy Body Dementia (LBD).
We’ve posted the webinar recording here —
If you listen to the webinar recording, please take LESS THAN FIVE MINUTES to answer six questions on our survey. See:
For additional information on the topics addressed during the webinar, see:
Orthostatic hypotension –
Parkinson’s – parkinsons.stanford.edu
Make an appointment with Dr. Santini at the Stanford Movement Disorders Center – 650-723-6469
Multiple System Atrophy – www.brainsupportnetwork.org/msa
Lewy Body Dementia – www.brainsupportnetwork.org/lbd
Our terrific volunteer, Denise Dagan, took notes from the webinar.
Orthostatic Hypotension (OH) in Parkinson’s, Multiple System Atrophy, and Lewy Body Dementia
Speaker: Veronica Santini, MD, movement disorders specialist, Stanford University
Host: Candy Welch, former MSA caregiver, Brain Support Network
September 18, 2017
Topics for this webinar are:
* Describe symptoms associated with orthostatic hypotension (OH) in
– Parkinson’s Disease (PD)
– Multiple System Atrophy (MSA)
– Lewy Body Dementia (LBD)
* List the conservative and medication interventions used for treatment
Normal Blood Pressure Response to Gravitational Change
Gravitational Change = changing from lying or sitting to standing, even climbing stairs. Gravity pulls blood into the legs and belly (up to 1 liter, or more). That means less blood goes to the heart, resulting in up to 20% less blood leaving the heart and consequent blood pressure decrease. Normally sensors in the neck see less blood pressure and sends signals to close blood vessels, increasing blood pressure. Important organs get nutrients and oxygen.
In OH the sensors are not working properly (baroreceptor reflex is dysfunctional), so blood vessels don’t close. They stay open and blood pressure drops, causing symptoms.
Common symptoms include: lightheadedness, dizziness, almost passing out, weakness, fatigue, visual blurring, headaches.
Less common are: buckling legs, walking difficulties, confusion, slowed thinking, shortness of breath, imbalance, jerking movements, neck pain/“coat hanger headache”, chest pain
Rare symptoms include: stroke-like symptoms, weakness or numbness, abnormal cramping/dystonia.
Evaluation of OH includes:
– History of autonomic symptoms
– “Orthostatic” blood pressure (BP) = measure BP in both laying and standing postures. OH is defined as a drop of the systolic >20 or diastolic >10
– Neurological examination
– Autonomic testing can be helpful in distinguishing PD/DLB from MSA
Approach to Treatment of OH:
Conservative therapy first, then adding Medications and, if necessary, Combination therapies (both conservative and medications, even a combination of medications)
Goals of Treatment:
1. Prevent loss of consciousness (this leads to falls and potential injury)
2. Prevent close calls (almost losing consciousness and)
3. Identify and prevent symptoms of OH (leg weakness, falls, somnolence, confusion)
4. Improve fatigue, exercise tollerance and cognition
Actions to Avoid:
– Standing motionless
– Standing too quickly
– Working with arms above shoulders
– Hot environments (anything that leads to sweating)
– High altitude
– Hot baths
– Dehydration !!!
– Vigorous exercise
– Fast or heavy breathing
– Large meals
– Straining with urination or defecation
– Coughing spells
– Water ingestion (60oz/day!)
– Salt tabs, dietary salt (chips, pretzels, nuts, deli meats, soups, tomato juice)
– Head of bed elevation 10-20 degrees/4” or 10cm (reduces postural change extremes, and urination)
– Physical maneuvers that raise orthostatic blood pressure (standing calf exertion, raise one leg on a step, knee bends, single knee kneel)
– Cooling vests, leg sleeves, binders around the abdomen after eating to prevent blood rushing to gut for digestion
Mineralocorticoid, a-1 agonist = woirks by expanding blood vessel volumes
Should be used carefully due to rise of volume overload, electrolyte abnormalities
Additional side effects: headache, swelling, weight gain, high blood pressure lying flat.
Peripheral z1 agonist = Works by squeezing blood vessels
Dose 5-10mg 3x daily
Common side effects: pupil dilation, goose flesh, tingling, itching
Can also cause high blood pressures when lying flat.
Droxidopa (Northera) (Newest FDA-approved Rx)
Norepinephrine (NE) pro-drug but the exact mechanism of action is unknown.
Studies have shown low standing NE
Dose 100-600mg 3x daily
Common side effects: headaches, dizziness, nausea, blurry vision, high blood pressure
Can also cause high blood pressures when lying flat.
Doctors advise against lying down when using all of these so you don’t raise blood pressure too high. Never take them before bedtime so blood pressure doesn’t go to high while sleeping.
Non FDA-approved Pharmacology:
Improves standing BP in patients w/OH
Does not increase BP when lying down
Effective alone or w/Midrodrine
Side effects: diarrhea, salivation, nausea, vomiting, muscle cramps, twitching\
a-2 adrenorectptor antagonist = increases norepinephrine and BP
Side effects: confusion, increase in heart rate, headache, or tremor
Regulation of supplements
QUESTIONS AND ANSWERS (all answers are by Dr. Santini, unless indicated)
Q: What can caregivers do to help?
A: Be the squeaky wheel by reminding your family member to keep hydrated, eat salty foods (even it that means the two of you eat different meals), help them check blood pressure throughout the day. Also, give your doctor a symptom report so he/she has a full picture of challenges at home. Doctors can’t fix what they don’t know about. Sometimes patients get used to having low BP, so they don’t report changes to their doctor. Caregivers can be more objective in how things used to be before BP issues arose, like seeing increased falls, more sleepiness, etc. Caregivers need the right amount of support, as well. Sometimes, the doctor can arrange for a nursing assistant to come into the home to do BP checks, or provide other services. Just let your neurologist know if you are feeling the least bit overwhelmed.
Q: How do you keep someone safe with OH without confining them to a wheelchair?
A: Doctors should make sure the patient’s BP is good enough to have a full and active life. It is a step-wise process, so be patient, but patients and their families or caregivers should be persistent. Make sure all aspects of the patient’s health influencing BP is investigated, the big picture is formed and all therapies possible are attempted.
Candy: We had a tilting wheelchair for my husband, who had MSA, so when he was feeling faint they could tilt the chair back making it easier for the body to maintain blood pressure, and preventing him from feeling awful or passing out.
Dr. Santini: Neurologists are often able to write a letter to your insurance company recommending such a chair so that it is covered by insurance. They are very expensive, but insurance did pay for Candy’s husband’s tilting wheelchair.
Q: How does blood pressure affect brain function?
A: There are several philosophies, but it is thought the blood carrying oxygen and nutrients doesn’t get to certain parts of the brain when BP is low. The most upper parts of the brain affect both thinking and leg function. Lack of oxygen and nutrients to these parts of the brain can cause all the symptoms mentioned; visual blurring, headaches, neck pain, dizziness, etc.
Q: Are there any new blood pressure (BP) treatments?
A: Yes, the newest is Northera. Anecdotal evidence shows it to be quite effective. But, the old ones are tried and true and new ones can be significantly more expensive.
Q: How do BP medications interact with Parkinson’s medications?
A: There are several issues here. Parkinson’s disease and atypical parkinsonian syndromes, like Lewy Body Dementia and Multiple System Atrophy cause problems with orthostatic hypotension. So, the disease itself causes OH problems. Almost every medicine doctors have to treat parkinsonian syndromes also drop blood pressure, unfortunately. Patients should understand they need not suffer. Let your physician boost your BP with some meds, then get your PD symptoms under control with other meds. It is more meds, but if it improves your quality of life because you can move better and you can think and not be dizzy, etc. it’s probably worth it. I frequently see patients who are not taking enough carbidopa-levodopa because it lowers BP. I boost the BP, then add enough carbidopa-levodopa to improve mobility. It’s a trade-off, but I feel quality of life is the most important thing while the patient is well enough in other ways to be active without feeling dizzy.
Q: Can beta blockers help?
A: With beta blockers you have to be careful. Beta blockers are often used for tremor control. We use those that don’t affect BP too much. They can be helpful for people who have very elevated heart rates. Usually, the best treatment is to use the BP boosting agents. Oftentimes, in the absence of Northera, which can sometimes cause an increased heart rate, if you treat BP, heart rate can come down.
Q: What foods and supplements are best for OH? Anything to be avoided?
A: It’s more how often you’re eating and how much you’re eating. The bigger the meal, the more your BP can drop afterward. If you are susceptible to BP drops after meals, an abdominal binder can be helpful. Put it on about 10 minutes before a meal and keep it on for an hour afterward. I recommend several small meals throughout the day, rather than three big meals. As far as what meals are best, we know some people have more difficulty with digestion of gluten or lactose. Try going gluten free first for a couple weeks to see if it makes a difference for you. If not, return the gluten and try going lactose free for a couple weeks. It’s a good test to see if you are one of those with these digestive issues.
Q: What do you do if you have both OH and hypertension?
A: This is by far the most challenging of the group. You have to decide on goals of care. Most commonly, people have hypertension, or high blood pressure, when they are lying flat. In that case you should avoid that flat position during the day. At night we sometimes give a short-acting high blood pressure medicine, something like captopril, clonodine, etc. It is more challenging when people have wide swings in BP. It is extremely common in MSA and advanced PD. Even standing or sitting people will have very high blood pressures, with systolic in the 180s of 190s. Others will have extremely low blood pressures standing, with systolic in the 70s of 80s and they are passing out. One thing doctors will do is ask patients to take their BP before they take the BP boosting medicine. Then, the doctor will advise against taking the BP boosting meds when BP is already high, but to take it later in the day. Sometimes, a person will need to avoid everything causing high BP. Sometimes not treating high BP is the best option, even though that would normally not be recommended. You have to treat which is causing the most symptoms and affecting quality of life.
Q: Can salt tablets help?
A: Yes, if you don’t like eating salt. Talk with your doctor. Taking a 1 gram tablet of salt in a tablet works better for some people than having salty meals.
Q: Can OH cause shortness of breath?
A: Yes! It’s a common symptom because the upper lungs aren’t seeing as much blood as they usually do when BP is normal. Gravity is pulling the blood down and those upper lung fields feel like they’re not breathing so people feel short of breath.
Q: Why does BP drop with exercise?
A: Sometimes it will raise, sometimes it will drop. You may notice basketball players wearing sleeves on their ankles and legs. Those are compression sleeves to help adjust BP. When we exercise, the blood vessels open up so all the blood flow can get to those muscles that are working so hard. The problem is that in OH we don’t have those extra reflexes to boost the BP back up. Sometimes vigorous exercise can drop BP in people who have OH. Those leg and arm sleeves can be very helpful in that case.
Q: Can OH lead to sudden death?
A: It is a more rare circumstance. It can certainly lead to heard dysfunction, and that could lead to sudden death. We know that in autonomic dysfunction people can also have arrhythmias, and that can lead to sudden death. If not exactly OH, sometimes it’s the autonomic failure that involves the heart that can lead to sudden death.
Q: Is OH more severe in MSA than in PD or LBD? Is treatment of OH different with these three diseases?
A: Treatment tends to be similar but you have to be ready as the patient, caregiver, and healthcare provider to accept more OH in MSA. OH is typically more severe in MSA than in PD or LBD. Sometimes very advanced PD or LBD (10+ years) may have severe BP swings, but MSA is more severe because OH occurs early in the disease course. BP swings/OH is one of the most prominent symptoms people have in the entire MSA disease course. Treatment goals in MSA may be different from PD and LBD as more accepting of BP swings.
These questions were sent in during the webinar:
Q: Someone has MSA w/OH but also supine hypotension (low blood pressure lying down).
A: This is easiest to treat because you just need to boost BP in all positions (lying down, sitting, and standing). I would be concerned something else is going on and would recommend autonomic testing to determine that.
Q: Someone has primary autonomic failure (PAF) with possible MSA. Does OH occur in PAF?
A: Oh, yes! This whole category of PAF is a difficult one. There is a current study looking at the natural history of primary autonomic failure. Based on that research they are finding some of these patients eventually meet qualifications for an PD or MSA diagnosis. Some people just have PAF, but not significant PD symptoms or progressive parkinsonism. The main symptoms these patients have is OH and they really suffer from that.
Candy: This is how my husband was diagnosed with MSA. First, doctors diagnosed PAF.
Q: Northera doesn’t help. Should I stop and restart it?
A: No. Sounds like you need to adjust the dosage. Tell your doctor. Sometimes, you just need to call or email, rather than make an appointment to see the doctor, for a medication adjustment. Sometimes, they may ask you to come in in order to understand the problem. If I had a patient report this to me and the patient was at the max dosage of 600mg/daily, I would cover all the bases with the patient. I would reassess everything, confirming that the patient is drinking enough water, eating enough salt, wearing compression stockings. Does this patient tolerate Florinef and, if so, can we retry it? Are you on an effective dose of Florinef or Midodrine, would adding pyridostigmine help the situation? When things get really tough, I sometimes temporarily reduces the anti-Parkinson’s medications (carbidopa-levodopa or dopamine agonist). Sometimes reducing the PD meds isn’t what’s necessary, but increasing carbidopa can reduce side effects of levodopa, sometimes. It’s worth looking at.
Q: Can coconut oil help OH?
A: Harmful? probably not, unless you have high cholesterol. Ask your doctor if you should or should not be eating coconut oil, based on your health numbers. There is no evidence that it helps. It’s just the new magic for everything.
Q: Questioner feels faint while having a bowel movement. What can be done?
A: Either urinating or defecating activates the opposite side of the autonomic nervous system, lowering blood pressure. People have passed out on the toilet. Bathrooms are dangerous with hard surfaces to hit your head on when passing out. The answer is to treat the constipation so there is no straining. Don’t treat to the point of diarrhea because there can be straining with that, as well. Any of these may help: Miralax, Senna, Cholase, or any stool softener. Another solution may be to put your feet onto a stool so knees are raised while on the toilet (Squatty Potty) can help defecation without straining. Massage the lower belly while trying to poop can help move your bowel. Best to poop after a hot meal and around the same time every day.
Q: What about SSRIs (antidepressants) for OH?
A: Yes, Prozac has been studied for use in OH. There is some research that it can help boost BP.
Q: Is Parkinson’s with OH more severe than PD without or a more rapidly progressing form of PD?
A: Everybody who has PD has a different form of the disease. I have heard the strangest symptoms that a neurologist would consider ‘off medication’ symptoms, or those not normally attributed to PD, but happen to be attributed to that person with PD. It’s very common for people with PD to have OH. They are just a little unlucky because with OH you get a lot of symptoms. Although you feel horrible and like you’re dying, sometimes, it doesn’t mean that your PD is more severe, just because you have those symptoms. It means it’s something we need to treat and get your quality of life better.
Q: It seems OH research is focused on MSA. Do you feel that is true, and if so, why?
A: Yes, it is very true because patients w/MSA have OH symptoms early and severely in the disease course. Researchers feel that if they can develop a treatment for OH in MSA, it will help those with PD. I feel more studies should be done for OH in PD because improvements in OH improves cognition and physical activity for patients with PD. Up to 30% of newly diagnosed PD patients have OH, so they would benefit from OH research.
Q: If I have severe OH, what kind of doctor should I see?
A: It depends on the specialty at different medical centers. If you come to see a movement disorder specialist at Stanford, I have had specialized training in treating OH. But, some movement disorder specialists prefer you see an autonomic specialist if you have OH. Other specialists who can treat OH include cardiologists or nephrologists. You just have to find the specialist most comfortable in treating OH at the medical center where you are being treated.
Q: Is OH caused by a pathology in the brain?
A: People with MSA, LBD and PD have an abnormal buildup of the protein alpha-synuclein in certain brain cells. These people can be affected by OH. Other atypical parkinsonisms, like PSP, CBD, etc. that don’t have alpha-synuclein don’t have OH so we feel there is a connection between OH and alpha-synuclein.
Q: Does Stanford have an autonomic testing center? Do you know where other autonomic testing centers are located in the US? What is the benefit of having this testing?
A: Stanford has a very good autonomic testing center. It is especially useful for people who have diabetes and PD, or in cases where symptoms seem more severe than what would be expected in PD so you would like to gather more information to determine if it is really MSA. For these people, it may be a good idea to have autonomic testing. Stanford is probably best place for autonomic testing on the west coast. Mayo Clinic in the midwest, and there are several places on the east coast are terrific, like Beth Israel.
Q: Some research shows that doctors see OH and automatically diagnose MSA. What’s happening here?
A: I see people newly diagnosed with PD who have some OH and they have been misdiagnosed with MSA. They actually have PD, but because PD medications lower BP, the medications can make their symptoms look more like MSA early in the course of their symptoms. When there is a question as to whether someone has PD or MSA, autonomic testing should be done to differentiate between the two. Seeing a movement disorder specialist rather than just a neurologist because they are specially trained to use set literature criteria that helps to differentiate between these conditions. The history of a person’s initial symptoms helps me figure out an accurate diagnosis. Also, seeing how a person’s symptoms progress helps to determine an accurate diagnosis.
Q: What does autonomic testing look like?
A: The patient lays flat on a special bed. There are several tests. In one they infuse a medication that causes sweating to see how autonomic nervous system responds. They may also have the patient do deep breathing to see if their heart rate and blood pressure responds correctly. They also suddenly change the patient’s position from lying to standing (by tipping the table up quickly) to see how heart rate and blood pressure system responds. Depending on the body’s responses to all these different tests, they can determine if they are normal or abnormal. If there are abnormal responses, it the problem coming from the brain or from the peripheral nervous system. That can be helpful in differentiating between disorders.
Q: What about Methotrexate?
A: That can be used if there is an immune component to the patient’s autonomic dysfunction.