Dysautonomia Conference 2020

Review of presentations, Dysautonomia International Conference 2020

Purpose 

The following list provides commentary on selected presentations from the Dysautonomia 2020 conference. I plan to add reviews of the "pending" presentations as they become available for viewing. I do not plan to watch 100 % of the presentations; the conference was quite lengthy. 

 

These talks will be temporarily available for viewing online, as of ___ (still not posted as of 11-13-20). See this blog post for the link to conference information, including how to register. Please do not contact me about how to sign up; I don’t know the details! 
 

It is essential to first have a basic understanding of dysautonomia and the autonomic nervous system in order to understand the content on this page.
Please start by reviewing this blog post and introductory webpage in order to learn what dysautonomia is and why it is so important to Sjogren’s patients.

Disclaimer  

My comments reflect my interpretation of the oral presentation. I did my best to relay what the presenter intended to say. I cannot verify the accuracy of the content. Please discuss any questions you may have about your conditions and any treatments with your neurologist, PCP, or other clinician providing care.

It is too big a task to provide links to information on every topic discussed, or even to define terms and common abbreviations. Please use a search engine as needed and refer the Dysautonomia International website.

   RECOMMENDED PRESENTATIONS:

  • Pain Management by P. Chopra

  • Exercise Therapy by N. Miranda

  • POTs/OI Workup by B. Goodman

  More to follow when released

Day 1:  October 15, 2020

Overview of Autonomic Disorders, Blair Grubb, MD

Pending- not viewed yet.

Overview of Orthostatic Intolerance (OI), Peter Novak, MD
 

OI, in short, means you feel awful upright, especially standing, due to abnormal control of heart rate (hr) and blood pressure (bp). There are many versions of OI, with POTs being the most common.  If you have dysautonomia symptoms that do not meet the classic POTS criteria, Dr. Novak suggests that you seek evaluation at a dysautonomia specialty clinic to determine the diagnosis.
 

POTS (postural orthostatic tachycardia syndrome) is defined by heart rate (hr) increase of 30 beats per minute (bpm) with 10 min. tilt table or standing test, 40 bpm if under age 19. Blood pressure (bp) is usually maintained. Note that later lecturers admitted that strict 30 bpm cutoff may not be appropriate in all cases.

BP occasionally falls with POTS, but usually not to the point of syncope (fainting). Fainting usually occurs when cerebral (brain) blood flow drops 40 % or more 
 

POTS differs from inappropriate sinus tachycardia, which exhibits high heart rate in all positions, including supine.
 

Hyperadrenergic POTS occurs when bp goes up w/ the tachycardia.

A condition called HCH, or hypocapneic cerebral hypoperfusion, occurs with low blood carbon dioxide levels in the blood. It is sometimes called “POTS without the tachycardia.” It is treated the same way. 

Comments about POTS treatment:  Unlike a few other experts, Dr. Novak recommended avoiding beta blockers for POTS unless elevated bp.
 

He did state that midodrine, fludrocortisone (Florinef), and pyridostigmine (Mestinon) were all helpful POTS medications.
 

OCHOS is orthostatic cerebral hypoperfusion syndrome which may be caused by small blood vessel disease, rather than dysautonomia. People with this often have trouble with fainting.

Overview of postural tachycardia syndrome, Satish Raj, MD, MSCI

Pending- not viewed yet.

Gastrointestinal Dysmotility in Autonomic Disorders, Laura Pace, MD, PhD

Pending- not viewed yet.

Day 2:  October 16, 2020

Small Fiber Neuropathies (SFNs), Kamal Chemali, MD
 

There is no consensus definition for SFN, other than it involves damage or die-off of the small unmyelinated and thinly myelinated nerve fibers in the peripheral nervous system.
 

80- 90 % of the autonomic nervous system (ANS) is innervated by these small fibers.

SFNs impacting sensations such as pain, light touch, temperature, and gut sensations often occur together with dysautonomia. 
 

Full autonomic testing:

  1. QSART  (sweating function)

  2. Cardiovagal (hr variability on deep breathing)

  3. Valsalva (hr, bp changes with bearing down, in this example, breathing against a tube)

  4. Tilt table test

  5. Skin biopsy

 

These tests support but do not rule out dysautonomia and SFN:

Skin biopsy positive in 50 %
QSART positive in 50 %
Together, skin bx and QSART pick up 75 % dysautonamias

 

Note: Negative skin biopsy does not r/o SFN, but helps if positive. The gold standard for diagnosis is clinical judgement.  
 

Causes: There are many causes of SFN:  diabetes, prediabetes ( these are the most common), autoimmunity esp. Sjogren’s, elevated triglycerides, high cholesterol, B12 deficiency, too much B6, alcohol, toxins, HIV and post viral.
 

Sjogren’s is #1 autoimmune disease associated with dysautonomia and SFN.  Others mentioned: SLE,  amyloidosis, RA, systemic sclerosis. 11-13 % of POTs patients have Hashimotos. Celiac disease also associated with POTS.  

Dr. Chemali Considers fibromylagia as part of the SFN/ dysautonomia spectrum. This coincides with Dr. Oaklander’s and Farhad's work at Harvard. This view is not shared among all neurologists.

Autoimmune Dysautonomias, Kamal Chemali, MD

These are very common, very underdiagnosed. Seen in many AI rheumatic diseases; often subclinical but measurable. TBD: Does it  lower quality of life, and if so how much?

Sjogren’s was a recurrent theme in this conference:

  • 60 % of Sjogren’s patients develop SFN

  • 50 % have  ANS dysfunction symptoms

  • 66 % have abnormal autonomic testing.

Dr Chemali, a neurologist, then went into an inaccurate explanation of how Sjogrens is diagnosed, but points for trying!  

Many dysautonomia specialists are learning to be on the lookout for Sjogren’s, because they may first present with neurologic features, especially SFN/ dysautonomia.

Sex Hormones and Dysautonomia, Svetlana Blitshteyn, MD

Lengthy review of menstrual cycle, and hormonal changes. 

Women tend to have first onset of dysautonomia symptoms with menarche (first period) and worsened symptoms during periods, menopause. Some reproductive age women do better with hormonal contraceptives (assuming they are otherwise appropriate).
 

I could not figure out a practical take home message other than women are more prone to dysautonomia, possibly due to the interaction of sex hormones with inflammatory pathways.

Gastrointestinal Dysmotility in Autonomic Disorders, Laura Pace, MD, PhD

Pending- not viewed yet.

Keynote:  Autonomic regulation of the immune system, Kevin Tracey, MD

Pending- not viewed yet  (I heard it was very good!)

Day 3:  October 17, 2020

Pain Management in Dysautonomia, Pradeep Chopra, MD
Recommend

Dr. Chopra covered a huge range of topics.  Some highlights:

Structural pain- caused by inflammation

Neuropathic pain- caused by nerve inflammation, can arise from structural damage

Small Fiber neuropathy (SFN) is caused by damage to the smallest nerves. Changes to temperature, touch, creepy crawly feeling, tingling

Autonomic neuropathy (dysautonaomia) is a type of SFN.   Many dysautonomias are caused by SFN, but they can arise in the central nervous system (e.g. concussion) or the ganglia, which are nerve “relay stations” located right next to the spinal cord. Dr. Chopra  listed the same causes as Dr. Chamali, under the SFN lecture, Day 2. 

Evaluation: History and physical, skin biopsy QSART, EMG (to rule out large fiber neuropathy)

Treatment SFN-

  1. Treat underlying cause:  diabetes, autoimmune disease, high cholesterol. Strict glucose control, including prediabetes, recommended.
     

  2. Symptomatic treatment

  • Amitriptyline, Nortriptyline-good results (my comment: drying!)

  • Gabapentin-not very good.

  • Duloxetine (Cymbalta)- too many of side effects, difficult withdrawal

  • Milnacipran (Savella)- similar to Cymbalta w/ better side effects (later presenter discouraged SNRIs like these if dysautonomia)

  • Topical cream (consider Ted’s pain cream) DO NOT use capsaicin, irritates nerves.

 

  3. Low dose naltrexone (LDN)1.5- 4.5 once a day, start at 0.5 mg

 

  4.Treat Mast cell activation syndrome when present. It causes inflamed nerves. 

Mast Cell Activation Syndrome (MCAS) refers to over activation of mast cells, a type of immune cell involved in allergic reactions. MCAS often co-occurs with dysautonomia, SFN, and many pain syndromes. The diagnosis is mostly based on clinical presentation, because testing can be difficult and inaccurate. Complex and controversial topic.
 

Treating MCAS can reduce pain of all types.

  • Antihistamines (drying) 

  • Ketotifen (compounded, hard to find) oral cromolyn.

  • Quercetin (OTC supplement) 500 mg twice a day.

  • Avoid trigger foods, air quality issue:  molds, toxins, fragrances

 

    5. Treat migraines which are very common in dysautonomia.

CGRP inhibitors such as Emgality often very helpful with few side effects.

Sequential compression boots ((Amazon) good for bad POTS/ headache day (must lie down to use)

 

    6. Correct neck balance/ cranial-cervical instability (Chiari, Ehlers Danlos Syndrome) -this can improve POTS.

 

     7. Treat muscle spasms/ pain.

  • Magnesium helpful. If can’t tolerate oral Mg (rec. chelated by Solgar) try Epson salt bath or topical creams (My comment: warm baths not good idea for POTS)

  • Small doses levo-dopa (Sinemet) may help
     

     8. Gut dysmotility : diarrhea, constipation, both, nausea, gastroparesis

Multiple medications mentioned He skimmed over treatments quickly, although highlighted Prucalopride (Resolor/ Montegrity) which can be hard to obtain in the US. (Unclear on the indication, but he was very enthusiastic about this drug).

 

         Mentioned low FODMAP diet, but did not go into.

 

    9. Treat central sensitization. Occurs with all chronic pain conditions, but especially neuropathic.

  • Be sure to have correct Vit. D and Calcium levels.

  • OTCs - alpha lipoic acid and N-acetyl cysteine  (doses not given)

  • Cannabis helps some. Avoid Hemp sources.

  • LDN (Low dose naltrexone): improves pain, fatigue, MCAS.  Takes a few weeks to see response. (item 3)

 

     10. Misc, treatments:  not commonly used, but may help:

  • IVIG

  • Low does ketamine nasal spray (off label, used in treatment resistant depression)  May cause nausea, vomiting, diarrhea.

  • Mg infusion

  • Ketamine IV (last resort)

  • Near Future:  Vagus nerve stim can reduce overall sympathetic activation
     

     11.  Restless Leg syndrome  

   L dopa, ½ pill bedtime(Sinemet 25/ 100)

Exercise Therapy as a Dysautonomia Management Tool, Nicole Mirananda, PT, DPT

Highly Recommend, even if you don’t have dysautonomia
 

First, as stated by multiple speakers:  Deconditioning does not cause POTS or other dysautonomias.  However, “being out of shape” makes these worse.

This presentation is phenomenal.  It explains why exercise therapy helps dysautonomia, and makes you feel better overall. The trick is to learn how to add in exercise that helps (this takes time to build up) without making your symptoms worse.

A few key points, but the practical aspects of this presentation are really worth watching.

  1. It is important to get some baseline control of symptoms before adding activity through multiple lifestyle measures and medication. 

  2. Exercise therapy usually fails due to inadequate support by trained professionals and unrealistic  goals. It is a trial and error process, requiring patience and determination.

  3. Support by professionals (PT, OT, others) who understand dysautonomia is very helpful.  This is tricky; there are not a lot out there. However, with this talk and resources, many patients can make progress. She provides numerous practical tips and resources.

  4. Realistic goals: May need to start off with as little as 30 seconds of activity. Core exercises are usually a good place to start). The CHOP protocol can be a helpful guide.

  5. Learning intuitively what works in very small/ incremental trials.  Need to figure out how to make exercise work for you without worsening symptoms. 
     

Other:

Explains how posture corrections often improve fatigue and dizziness.  Usually need to work with PT on this.  

Keynote:  Autonomic regulation of the immune system, Kevin Tracey, MD

Pending- not viewed yet  (I heard it was very good!)

Expert Q and A:  Clair Fancomano MD,  Brent Goodman, MD, Lauren Stiles, JD
 

An interesting and roving discussion. Here are a few things that caught my attention:

  1.  Most patients with dysautonomia experience brain fog.

  2. The problem with complex diseases:  Need for multidisciplinary teams. This rarely happens. Having a PCP who is interested and willing to learn more can be helpful.  

  3. POTS may be an autoimmune disorder, but we still don’t understand the cause.

  4. Concussion often triggers POTS or dysautonomias. Even though people feel “dizzy”, vestibular testing is almost always normal.

  5. Much misc. on EDS (Ehlers Danlos syndrome)

Day 4:  October 18, 2020

Autoimmune Autonomic Ganglionopathy (AAG), Steven Vernino, MD, PhD

I found this fascinating, but AAG is a rare cause of dysautonomia. He discussed the difference between AAG, autonomic failure, and POTS. They have many symptoms in common, but different causes. Treatments are similar.  

The POTS/ OI Workup: What should we screen for?  Brent Goodman, MD
Recommend
 

This is an excellent presentation, although much of the content about diagnosis and treatment was covered in earlier presentations. The November 7, 2020 virtual Sjogren’s Foundation conference presentation is more concise and focuses specifically on Sjogren’s and dysautonomia. I highly recommend watching that if you get a chance. 
 

Many unknowns on these topics: mechanisms, genetics, biomarkers, long term natural history.  No consensus on approach to evaluation. 
 

Important features of evaluation:

  1. Establish diagnosis

  2. Onset/ trigger

  3. If POTs, subtype

  4. Extent of impairment

  5. Identify comorbidities such as diabetes, AI diseases, vitamin def (esp B 12),  Mast cell activation  (gave list of tests)  

  6. Use these as a platform of treatment   (autoimmune vs diabetes vs concussion as cause all call for different approaches)
     

Diagnostic evaluation may need to be ongoing, repeated for changes in clinical status.  

Tests (do not necessarily need all)

  • Autonomic tests such as Tilt table test, QSART

  • GI motility testing (not sure exact tests),

  • Skin biopsy SFN, lip biopsy if Sjogren’s suspected and SSA negative, ECG/ echocardiogram, thyroid, CBC, supine/ standing catecholamines

  • EDS/ joint mobility tests

  • Tests for viral infection?? HIV, hepatitis, EBV  (not sure if he specified)


Mimics
Mast cell activation (MCAS) which is often comorbid, anemia, adrenal insufficiency, pheochromocytoma, thyroid disease, PTSD, weight loss esp. due to eating disorders/ and chronic volume depletion. 

 

Misc. highlights

Gastrointestinal manifestations of dysautonomias:

  • Swallowing dysfunction/ esophageal dysmotility are VERY COMMON in POTS  

  • Abdominal pain/ bloating may be due to gastroparesis.
    In the context of dysautonomia and autoimmune diseases, DO NOT attribute GI symptoms including constipation and diarrhea to “irritable bowel syndrome” which he expresses great skepticism of being an actual entity, but rather another term that people use when they don’t understand symptoms and would rather write it off as a functional disorder.
     

MCAS

30 % with POTS test positive MCAS.
 

POTS/ MCAS/ Sjogren’s/ Migraine combo is common.  MCAS–dysautonomia relationship is not fully understood.  There are many overlapping features of Dysautonomia/ MCAS.

 

Blitz review of MCAS symptoms:  Hives, itchy skin, flushing face and chest, diarrhea/ bloating, postural hypertensions, bladder pain/ frequency / urgency without UTI (may be a cause of interstitial cystitis)

Recurrent anaphylaxis, ? bone pain ? hair loss

Reviewed tests, which are sometimes normal.  
 

My NOTE:  see YOUTUBE presentations by Dr. Ann Maitland for better description MCAS. 

https://www.youtube.com/watch?v=LddITjni4G0

Autoimmunity in POTS, Artur Fedorowski, MD

Extremely academic and dense; don’t recommend for patients. 

Non-Pharmacological Management of POTS, Tae Chung, MD

Much basic review of hydration/electrolytes/ exercise, lifestyle changes. Repeated the same points as the excellent presentation by Nicole Miranda on day 2. Only addition was he suggested getting an electronic heart rate/ blood pressure monitor.

Pharmacological Management of POTS, Satish Raj, MD

The basics of treatment have been repeated many times in the conference. Not all presenters agree on medical management, especially when it comes to Beta blockers and DDAVP (desmopressin).

  1.  POTS is not just tachycardia, patients feel awful when upright.  Plasma volume is usually low, typically 15 % lower than it should be when adjusted for body size. 
     

  2.  Concepts around POTS medications/ treatments:
     

Agents that increase blood volume:

  • Water, salt supplements (many products discussed; Liquid IV is a favorite)

  • Fludrocortisone

  • IV saline- 1-2 liters can help a lot but temporary

  • Acute DDAVP treatment helps, often with remarkable benefit for part of the day.  Can cause low sodium levels. Tab or nasal spray.  Dr Raj likes to reserve for special events, can help you feel ok for 3-4 hours.   Vs. Blair Grubb, 0.2 mg at bedtime with routine sodium levels.  

 

Hemodynamic agents:

  • Pyridostigmine (Mestinon)- side effects: diarrhea, salivation (good!) ,nausea

  • Midodrine

  • Beta blocker: Propranolol (low dose, 4 x/ day)  Many don’t tolerate due to low stroke volume (amount of blood entering the heart) due to low blood volume. Use low dose to start

  • He likes propranolol and midodrine as first line. Propranol controversial among specialists.

 

OTHER comments on POTS treatments:

Anything that includes NE reuptake inhibitors:  not good, rev up sympathetic nervous system, may increase heart rate.  (Does not like SNRIs) Also controversial; other specialists do use.

              SSRIs (Zoloft etc) may help.  

Avoid verapamil: dilates blood vessels 

Sub Q IG : early data looks good need more studies.


Brain fog:  Modafinil used off label in POTS, etc.  Helps some, seems to increase resistance to fatigue. Other stimulants discussed, but concern re stimulate sympathetic nervous system.

Research Updates:  Rafaello Furlan, MD and Franca Dipaola, MD

Pending- not viewed yet.

Data Blitz: POTS Research updates (panel)

Pending- not viewed yet.

Expert Q and A, Closing, Vernino, Pace, Stiles
 

POTS is not just tachycardia. Brain fog is a huge problem. Some patients have orthostatic intolerance that looks like POTS, but do not meet the cutoff of heart rate rising 30 bpm with standing. This POTS cutoff is probably artificial; need for biomarkers to diagnose the condition.
 

POTS often associated with insomnia, hypersomnia
 

Why don’t we have a better understanding of POTS and other dysautonomias?

  • Stigma:  Dysautonomias are often labeled as “functional disorders.” Even neurologists who specialize in dysautonomia can be thought of as quacks which is rather disturbing, given that this is a highly specialized academic field.   

  • Massive need for MD education, neurology fellowships in in the autonomic field.

  • Silo problem:  complex disorders can’t be managed in silos.  GI specialists and rheumatologists are often not trained re dysautonomia. Sjogren’s researchers are often not talking with autonomic researchers.  The overlap is immense. 
     

Many COVID patients are developing POTS.  Sadly, the onslaught of new patients is providing an opportunity for more robust research, helping us get a better handle on the probable autoimmune mechanisms behind these conditions.  

Updated 11-13-20