An article just published by Dr. Horacio Kaufmann and Dr. Jose-Alberto Palma in Movement Disorders Clinical Practice reviews the frequency, diagnosis and treatment of orthostatic hypotension, a common problem in patients with Parkinson disease, dementia with Lewy bodies and multiple system atrophy. Here is a summary of the main sections of the article:

Orthostatic hypotension, i.e., low blood pressure when standing up, can reduce the supply of blood to organs above the heart, most notably the brain, resulting in different symptoms. Symptoms can be very disabling, have a profound impact on quality of life, and increase hospital admissions.

How is orthostatic hypotension defined?

Orthostatic hypotension (OH) is defined as a sustained fall in blood pressure (BP) when standing up. The current definition of OH is a fall of at least 20 mmHg in systolic BP or 10 mmHg in diastolic BP within 3 minutes of standing or upright tilt.

How frequent is orthostatic hypotension in people with Parkinson disease or multiple system atrophy?

An estimated 30 to 50% of people with Parkinson disease (PD) have orthostatic hypotension. The prevalence of orthostatic hypotension in PD increases with age and disease duration. Although the prevalence of orthostatic hypotension in PD is relatively high, not all people have symptoms. In a recent study of 210 people with PD, only 16% of people had symptomatic orthostatic hypotension. In that study, symptoms of orthostatic hypotension appeared when the subject’s mean blood pressure dropped below 75 mmHg in the standing position. In multiple system atrophy (MSA), orthostatic hypotension appears in 70-80% of subjects.

Orthostatic hypotension can be the first problem in people with PD, dementia with Lewy bodies or MSA and appear several years – or even decades – before the onset of the typical motor problems of the disease. Therefore, people with orthostatic hypotension without significant motor or cognitive deficits, should be closely monitored to detect early signs or symptoms of PD or MSA.

What are the symptoms of orthostatic hypotension?

Orthostatic hypotension can appear with or without symptoms. Not all people with orthostatic hypotension have symptoms. People with PD and orthostatic hypotension can tolerate very low BP when standing with only mild or no symptoms at all but fainting can occur if other circumstances are added (e.g., large meals, alcohol, very warm weather, dehydration, or medications to lower the blood pressure).

The typical symptoms of orthostatic hypotension are lightheadedness, dizziness, blurry vision, and, when the drop in BP when standing up is pronounced, loss of consciousness (fainting or syncope). Symptoms occur only when standing, less frequently when sitting, and abate when lying down. People with orthostatic hypotension may also complain of generalized weakness, fatigue, leg buckling, occipital headache, neck and shoulder (“coat hanger”) discomfort, and shortness of breath due to ventilation/perfusion mismatch in the lung.

Symptoms of orthostatic hypotension typically disappear after the subject sits or lies down because blood flow returns back to the brain thanks to gravity.

Symptoms of orthostatic hypotension can be non-specific, including fatigue and difficultly concentrating and may sometimes mimic a levodopa “off” motor state in people with PD. In these cases, the diagnosis of orthostatic hypotension may be missed unless BP is measured in the standing position. It is important to realize that postural lightheadedness mimicking orthostatic hypotension may be caused by other problems, such as vestibular dysfunction, balance problems or other movement disorders.

Symptom severity often varies from day-to-day and fluctuates throughout the day. The morning hours tend to be most difficult as orthostatic hypotension symptoms are aggravated by overnight urination, which is very common in people with PD. Meals, particularly those rich in carbohydrates and sugars, cause drops in BP after meals. This is referred to as post-prandial hypotension.

In general, people with PD and MSA should be screened for orthostatic hypotension, even if they have no symptoms.

What causes orthostatic hypotension?

Orthostatic hypotension is a relatively frequent problem in the general population, particularly in the frail elderly, and can be due to a variety of medical conditions, such as intravascular volume depletion (e.g., severe dehydration), varicose veins, severe anemia, or medications that lower the blood pressure (known as anti-hypertensives). In theses cases, orthostatic hypotension usually improves significantly or resolves after these causes are treated.

In people with PD, orthostatic hypotension is due to problems in the release of the neurotransmitter norepinephrine from the nerves arriving at the blood vessels. These nerves control vasoconstriction when we stand up and maintain the BP stable. In people with PD and related disorders, the neurotransmitter norepinephrine is not released properly from the nerves, and the blood vessels do not constrict when needed, resulting in low BP. When orthostatic hypotension is caused by problems in norepinephrine release it is referred to as neurogenic orthostatic hypotension.

How is neurogenic orthostatic hypotension diagnosed?

The diagnosis of orthostatic hypotension requires BP readings while flat and standing up. This can be done regularly, or with the help of a tilt-table test. A fall upon standing up of at least 20 mmHg in systolic BP or 10 mmHg in diastolic BP is required to make the diagnosis of orthostatic hypotension.

In some people with PD, the fall in BP may not occur every time they stand up. In these cases, the use of ambulatory BP monitors to measure the person’s BP every 30-minutes for a whole day, can assist in the diagnosis and management of orthostatic hypotension.

On the other hand, some people with PD can experience symptoms suggesting orthostatic hypotension but a significant fall in BP cannot be documented during office visits and the ambulatory 24-hour BP is normal. This has been recently described as “inebriation-like syndrome”, although the cause is unclear. Misdiagnosing orthostatic hypotension in people who do not have it can lead to unnecessary medications that have side effects.

How is orthostatic hypotension treated?

Orthostatic hypotension can be treated. The goal of treatment is not achieve normal BP values, but to reduce symptoms of orthostatic hypotension and the improve quality of life. The steps in management include: a) correcting aggravating factors, b) non-pharmacological treatment and c) pharmacological treatment.

1. Correction of aggravating factors

This include stopping medications that can lower BP, such as diuretics, anti-hypertensives, some medications used for prostate hypertrophy and urinary symptoms, medications for erectile dysfunction, medications for angina, some antidepressants. Levodopa (Sinemet®) can also lower the BP and adjusting its dose may be necessary in people with PD and orthostatic hypotension. Anemia (low hemoglobin in blood) can worsen orthostatic hypotension and should be investigated and treated accordingly.

2. Non-pharmacological measures

Symptoms of orthostatic hypotension can be improved with time, patience and non-pharmacologic changes. It is tempting to try to control OH only with medications. However, these are not effective enough and may have adverse effects. Treatment of orthostatic hypotension is more successful if non-pharmacologic measurements are implemented first.

Following is a series of steps to improve symptoms of OH. All steps may be implemented at the same time. If performed properly, these can lead to a dramatic improvement, even with no medications.

• Liberalize water intake. People with orthostatic hypotension need more water than healthy people. People with orthostatic hypotension should be drinking 3 quarts/day (~2.5 liters). Ideally, the best is to drink water and supplement it with salt (see the next point). Tea and coffee increase urine output so, at the end, they may worsen your symptoms. Diet beverages are also acceptable. Sports drinks, juices, and non-diet beverages are not recommended due to their high-sugar content (see below) Diet (sugar free) sport drinks are fine.

 

• Liberalize salt intake. Add as much salt to your meals as you can handle. Increasing salt in your meals will help increase your blood pressure. Most of people do not need to take salt tablets, which may cause abdominal discomfort in some people.

 

• Wear compression stockings (also known as TED stockings). This can be found in medical supply stores. Compression stockings will reduce the venous pooling that occurs when standing up and, therefore, will increase your blood pressure when standing. There are several strengths for stockings. You can try first a medium strength (i.e, 20-30 mmHg). To be useful, compression stockings should be worn up to the abdomen. Stockings up to the knee only are not effective. You do not need to wear the stockings while sleeping.

 

• Wear an abdominal binder (i.e., a Velcro belt around your belly). This can be found in medical supply stores. The mechanism is similar to that of the compression stockings. You do not need to wear it during sleep.

 

• Sleep with the head of the bed raised at least 30 degrees (ideally 45-50 degrees). This is useful because people with orthostatic hypotension frequently have supine hypertension (i.e., high blood pressure when lying down). To avoid supine hypertension, people should never lay flat. Sleeping with the head of the bed raised will also reduce the urine output overnight, making you wake up less times to urinate at night, and will improve your blood pressure in the morning. The best way to raise the head of the bed is to get an electric bed or an electric mattress. These are commercially available in several sizes. Other, less efficacious ways to increase the head of the bed are using a wedge, or just by putting some books/bricks under the upper feet of the bed.

 

• Drink 500 ml of cold water 30 minutes before getting out of bed in the morning. This will increase your blood pressure when you get up. Drinking 500 ml of water in any other moment of the day will also increase your blood pressure. You may use this on an as needed basis (but make sure you drink, in total, around 3 quarts/day of liquids)

 

• Start a physical therapy program. In people with orthostatic hypotension,
  hysical exercise will decrease blood pressure. But exercise is crucial to keep muscles active. Therefore, in order to avoid low blood pressure when exercising, people should perform recumbent exercises (e.g., recumbent bicycle, elastic bands, rowing machine, etc.). The best exercise is, by far, the one performed in a swimming pool. This is because the hydrostatic pressure of the water will prevent the fall in blood pressure. Therefore your blood pressure will not fall so dramatically if you are inside the water (with the head out, of course, so that you can breathe) even in spite of the fact that you are standing. While you are inside the water you will feel much better and you will be able to exercise without symptoms. The better your physical shape is, the less intense your symptoms of orthostatic hypotension will be. Therapies such as yoga, tai-chi, or similar are highly advisable too.

 

• The following factors worsen orthostatic hypotension (i.e., decrease blood pressure) and should be avoided (or can be used right before going to bed to lessen supine hypertension during nighttime):
  1. Hot and humid temperatures
  2. Physical exercise (see above)
  3. Dehydration (see above)
  4. Alcohol
  5. High glycemic index carbohydrates. Try to reduce high-glycemic carbohydrates in your meals meals. Also try to have several, small meals (5-6) instead of the three traditional meals.

 

• These are high-glycemic carbohydrates that you should reduce/avoid: Potatoes, yams, candy/sweets, bagels, white bread, white pasta, pizza, corn, rice, rice cakes, oatmeal, wheat, grits, cereals (corn flakes, etc.), soft drinks, bottled juice (orange, apple, etc.), cakes, cookies, ice cream, chocolate, full fat milk, watermelon, bananas, grapes, rye, yogurt, corn syrup, and maple syrup.

 

• Try to increase low-glycemic index carbohydrates in your diet, including: Whole-wheat bread, whole-wheat pasta, brown rice, pearl barley, skim milk, reduced-fat yogurt, apples, grapefruits, oranges, pears, peaches, just-squeezed juice, prunes, beans, black-eyed peas, chickpeas, peas, hummus, lentils, soybeans, cashews, peanuts, carrots, diet soda, almonds, nuts, quinoa, and olives.

 

• Be aware of your symptoms. If you experience symptoms of orthostatic OH, you will find relief by performing physical counter-maneuvers (making a fist, crossing your legs, clenching your buttocks), useful to increase your blood pressure when you are standing. If these counter-maneuvers are not enough, please, sit or lie down quickly to avoid fainting and get injured.

3. Pharmacological treatment

While non-pharmacological methods are very effective when performed properly, many people with orthostatic hypotension still require pharmacological treatment to improve symptoms. Two complementary strategies are used: a) Long-term increase in water and salt retention with fludrocortisone and b) Short-term increase in BP with midodrine or droxidopa.

For patients in whom these treatments are not effective, we also have clinical trials.

Click here to find out more about clinical trials for patients with multiple system atrophy and also Parkinson disease with orthostatic hypotension: https://dysautonomiacenter.com/2017/04/10/two-new-studies-open-for-patients-with-msa/

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Read the paper here: http://onlinelibrary.wiley.com/doi/10.1002/mdc3.12478/full