“Neurogenic Orthostatic Hypotension: Diagnosis, Management, and Research” – Lecture Notes

“Neurogenic Orthostatic Hypotension: Diagnosis, Management, and Research” – Lecture Notes

The Multiple System Atrophy Coalition hosted a two-day conference in late October on various aspects of multiple system atrophy (MSA), which is a disorder similar to Parkinson’s disease (PD). (MSA is called an atypical parkinsonism disorder.) One of the talks, presented by Katie Kompoliti, MD, a movement disorder specialist at Rush University, focused on neurogenic orthostatic hypotension (nOH), which can occur in PD as well. Neurogenic orthostatic hypotension refers to a neurological disorder causing a large decrease in blood pressure when a person stands after sitting or lying down. 

Dr, Kompoliti described the roots of nOH, as well as its diagnosis. She noted these symptoms of nOH:

  • Non-specific symptoms: generalized weakness, lethargy, fatigue, falls, nausea, leg buckling
  • Cerebral hypoperfusion: dizziness, lightheadedness, presyncope (loss of consciousness), cognitive difficulties, headache
  • Retinal hypoperfusion: blurred vision
  • Muscle hypoperfusion: neck and shoulder pain (“coat hanger” headache)
  • Myocardial hypoperfusion: angina
  • Lung hypoperfusion: orthostatic dyspnea

The speaker also listed medications and therapies that can help with managing the debilitating symptoms of nOH.

If you are dealing with nOH and PD, you may want to watch this webinar. To do that, you must first register for the conference and be logged in. Go here and click “Register Now.” Follow the instructions and email link, then return to the conference website. If you are logged in, you can access this link to view the recording.

Here are the slides for Dr. Kompoliti’s talk: (at this time, these 35 slides can *only* be viewed or downloaded if you are logged in to the conference).

Stanford Parkinson’s Community Outreach has a webpage listing lots of resources on orthostatic hypotension in PD.

Note in particular the Stanford/Brain Support Network webinar on OH in PD, MSA, Lewy body dementia, and other disorders here.

I listened to this presentation in late October and am sharing a summary of the talk below. For many of you, just reading the summary is sufficient.

– August Besser

Neurogenic Orthostatic Hypotension: Diagnosis, Management, and Research – Lecture Notes

Speaker: Dr. Katie Kompoliti, Professor of Neurology at Rush University Medical Center

Conference Host: Multiple System Atrophy Coalition

October 23, 2020

Summary by August Besser, Stanford Parkinson’s Community Outreach

Blood Pressure Control

  • Blood pressure is controlled by theautonomic nervous system, which is broken down into:
    • Sympathetic nervous system: raises blood pressure; also known as “fight and flight” reflex
    • Parasympathetic nervous system: lowers blood pressure; also known as “rest and digest”
  • The autonomic nervous system 
    • Depends on good function of an elaborate network of receptors, nerves, hormones
    • Affects everything in the body

Orthostatic Hypotension

  • A sustained decrease in the systolic blood pressure of 20 mm Hg OR a decrease in diastolic blood pressure of 10 mm Hg WITHIN 3 minutes of standing WHEN compared with blood pressure when sitting or in the supine position
  • Orthostatic hypotension can be caused by: neural-mediated hypotension (NMH), low blood volume, drug side effects, aging

Neurogenic Orthostatic Hypotension (nOH)

  • nOH affects the central nervous system: multiple system atrophy, spinal cord injury
  • nOH also affects the peripheral nervous system: Parkinson’s disease, dementia with Lewy bodies, pure autonomic failure, neuropathies

Symptoms of nOH

  • Non-specific symptoms: generalized weakness, lethargy, fatigue, falls, nausea, leg buckling
  • Cerebral hypoperfusion: dizziness, lightheadedness, presyncope, syncope (loss of consciousness), cognitive difficulties, headache
  • Retinal hypoperfusion: blurred vision
  • Muscle hypoperfusion: neck and shoulder pain (“coat hanger” headache)
  • Myocardial hypoperfusion: angina
  • Lung hypoperfusion: orthostatic dyspnea

Screening Questions for Suspected OH Case

  1.  Have you fainted/blacked out recently?
  2. Do you feel dizzy or lightheaded upon standing?
  3. Do you have vision disturbances when standing?
  4. Do you have difficulty breathing when standing?
  5. Do you have leg buckling or leg weakness when standing?
  6. Do you ever experience neck pain or aching when standing?
  7. Do the above symptoms improve or disappear when you sit or lay down?
  8. Are the above symptoms worse in the morning or after meals?
  9. Have you experienced a fall recently?
  10. Are there any other symptoms you commonly experience when you stand up or within 3 to 5 minutes of standing, and get better when you sit or lay down?

Lying Down to Standing Up

  • Supine: Blood volume is distributed evenly through the body
  • Move to upright position: causes a shift of > 500 mL of blood to the lower body
  • Autonomic nervous system response: Baroreceptor reflex activates the sympathetic nervous system
  • Healthy subject: normal response- nerves release signals for blood pressure and heart rate increases
  • Patient with orthostatic hypotension: insufficient norepinephrine response due to autonomic nervous system difficulties; inadequate blood pressure and heart rate

Daytime to Nighttime Blood Pressure Changes

  • Normal blood pressure: small changes and variation throughout the day
  • OH blood pressure: peaks and valleys; high spikes akin to a “roller coaster”
  • Eating food can result in changes to blood pressure; the blood pools in in the abdomen region
  • OH patients have big peaks in BP when eating
  • When going to bed, the extreme change in BP when lying down can result in passing out; the same peak occurs in the morning

Orthostatic Hypotension Monitoring

  • In-clinic monitoring: blood pressure test
  • At-home monitoring: BP or heart rate test
  • Medication review: reduce or modify medications when possible
  • Evaluate causes of orthostatic hypotension: review the patient’s cardiac history
  • Specialty testing: autonomic testing, plasma catecholamines, 24-hr ambulatory BP monitoring, “sweat test”

Treatment for Orthostatic Hypotension

  • Conservative, non-pharmacological measures
  • Pharmacological treatment

Lifestyle and Meals

  • Avoid: hot weather, hot showers, prolonged bed rest during day, physical immobility
  • Encourage: seated exercise (rowing or stationary bike), swimming
  • Avoid: high carb meals
  • Encourage: frequent smaller meals
  • Avoid: day time alcohol

Glycemic Index

  • Avoid: high carb foods; take in less quantity

Volume Expansion

  • Avoid: alcohol, caffeine, sugary beverages
  • Encourage: increased fluid intake (2.5 liters per day)

Physical Counter Maneuvers

  • Encourage: changing positions gradually, briefly sitting before standing
  • Avoid: straining with closed glottis (Valsalva maneuver)
  • Encourage: leg crossing, standing on tip toes, stooping, squatting, buttock clenching

Compression Garments

  • Encourage: high-waisted compression stockings of 15-20 mm Hg compression
  • Avoid: knee-high stockings (cause a placebo effect)
  • Encourage: use of an abdominal binder

Ameliorate Supine Hypertension

  • Elevate the head of the bed 30-40 degrees
  • Alleviates the dramatic drop in blood pressure

Medications for Orthostatic Hypotension

  • FDA approved: Midodrine and Droxidopa
  • Not approved (open label): Atamoxetine, Fludrocortisone, Pyridostigmine

Volume Expansion

  • Florinef: increases water reabsorption, intravascular volume, and blood pressure (in all positions)
    • Doses higher than 0.2 mg / day do not improve therapeutic effects; should be used for short periods
    • Side effects: ankle edema, low potassium, potential for left ventricular and renal failure with long term use

Sympathetic Enhancement

  • Medications:
    • Midodrine: short-acting (3 hours), peak effect after 1 hour
    • Droxidopa: short-acting, peak effect after 3.5 hours; best response in patients with low norepinephrine
    • Atomoxetine: best response in patients with high norepinephrine; patients with MSA would be the most suitable
    • Ampreloxetine: longer-acting than Atomoxetine; currently in phase 3 trials
  • How they work: Norepinephrine stimulates nerve endings at blood vessels; the blood vessels tighten and blood pressure increases

Lab Test for Orthostatic Hypotension

  • Check plasma norepinephrine levels
    • Patients with MSA have high levels
  • PD patients have a good response to Droxidopa

Other Medications for OH

  • Pyridostigmine: enhances cholinergic transmission
    • Modest effect when used alone
    • Synergistic effect when used with Midodrine or Atomoxetine