Different Types of Dysautonomia

If you’ve ever heard of postural orthostatic tachycardia syndrome (POTS) or neurocardiogenic syncope (NCS), you have probably heard of dysautonomia. However, unlike POTS and NCS, dysautonomia does not refer to a single medical condition. Rather, it is the collective term for a group of medical conditions that affect a person’s autonomic nervous system. POTS and NCS are two types of dysautonomia, but dysautonomia is not limited to POTS and NCS.

Dysautonomia does not have a “cure,” which makes raising awareness about these conditions critical. These conditions are frequently misdiagnosed^[1], and many doctors are either unaware of these conditions or simply do not believe in their existence. Given these concerns, it’s easy to understand why it’s also important to educate people about the various types of dysautonomia – it can help people better understand what they’re facing and can help those who do not have these conditions understand them better.  

If you’ve never heard about the various types of dysautonomia, you’re likely not alone. There are numerous different types of this condition, all varying in terms of occurrence in the population. Some of these types include:

Neurocardiogenic Syncope (NCS)

NCS is the most common form of primary dysautonomia, affecting tens of millions of people around the globe^[2]. NCS can be both mild and severe. People with mild NCS will likely experience nothing more than one or two instances of fainting in their lifetime. However, those with severe NCS can faint multiple times a day, putting them at risk of head injuries, falls, and broken bones and severely affecting their regular life. 

Orthostatic stress – that is, standing upright – is the most common trigger for NCS. Other triggers can include dehydration, stress, alcohol, and heat. NCS is also known as vasovagal syncope (VVS).

POTS

Like NCS, POTS is another relatively widespread form of dysautonomia. It affects between 1 and 3 million in the United States^[3], and about 50% of reported cases are female. POTS can also be a secondary condition that people with autoimmune disorders develop. However, secondary POTS is a result of the primary condition and can vanish when that condition is treated, unlike primary POTS.

POTS causes blood circulation problems and can result in a rapid heart rate (especially when you stand up), chest pain, shortness of breath^[4], lightheadedness, fainting, shaking, stomach upsets, and more.

Familial Dysautonomia (FD)

FD is an extremely rare form of dysautonomia, which primarily affects people of Ashkenazi Jewish heritage^[5]. Within this community, it is more common, affecting 1 out of every 3700 Ashkenazi Jewish people. 

Symptoms of FD generally appear in infancy and childhood. These can include difficulty regulating body temperature, delayed development, poor balance, bed wetting, lung infections, and heart and kidney problems. Unfortunately, the condition is usually fatal by the time a person reaches their thirties^[6]. It isn’t always fatal – some people have lived into their 70s – but this is a very unlikely outcome. Prenatal screening, testing and genetic testing of the parents is reducing the incidence of FD.

Diabetic Autonomic Neuropathy (DAN)

Unlike the conditions discussed so far, diabetic autonomic neuropathy is not a primary dysautonomia. Rather, it is a secondary dysautonomia associated with diabetes. If a patient’s diabetes is treated effectively, their DAN can improve as well.  

However, it is included in this list because of its rate of occurrence. While NCS is the most common form of primary dysautonomia worldwide, DAN is the most common for dysautonomia of any type. A serious complication of diabetes, it can lead to an increased rate of heart issues and death from cardiovascular diseases.

Symptoms include:

• Breathing problems
• Constipation and gastroparesis
• Erectile dysfunction
• Fast resting heart rate
• Low blood pressure when standing
• Impaired neurological function

Multiple System Atrophy (MSA)

Another rare form of dysautonomia, MSA is a form of dysautonomia that usually develops in people over the age of 40. Symptoms include motor difficulties, balance problems, and speech issues. 

Because of its similarity to Parkinson’s disease, MSA is often misdiagnosed. Multiple system atrophy is usually life-threatening, and most patients are confined to their beds within 2 years of their diagnosis. This condition is generally fatal within 5-10 years of diagnosis.

There is little known about what causes MSA, and there is no cure or course of treatment available at the moment. However, some symptoms can be managed with lifestyle changes and medication.

Pure Autonomic Failure (PAF)

Previously known as Bradbury-Eggleston Syndrome^[7], PAF results in the deterioration of the cells of your autonomic nervous system, ultimately causing dysfunction of the autonomic nervous system. 

Orthostatic hypotension – that is, feeling dizzy, fainting, and having visual problems, tiredness, and chest pain upon standing – is a key symptom of PAF. This is caused due to a fall in blood pressure when you stand, and the symptoms can be relieved by sitting or lying down.

PAF generally occurs in middle age, and it affects more men than women. However, it can be seen in younger patients as well. PAF can also result in a lower risk of heart attacks and strokes^[8].

Autonomic Dysreflexia (AD)

AD is another form of secondary dysautonomia and is associated primarily with spinal cord injuries^[9]. It can also occur as a result of Guillain-Barré syndrome, severe head trauma, and as a side effect of some medications and stimulants like cocaine. 

This condition involves the involuntary overreaction of the autonomic nervous system to stimulation. Generally, it occurs when there is an irritant affecting the person below their spinal cord injury. As they are unable to properly process the messages that their body is receiving, they experience a sharp rise in blood pressure. Other symptoms include excessive sweating, muscle spasms, and a change in heart rate. Unless properly managed, an AD attack can lead to a stroke.

Baroreflex Failure (BF) 

BF is a rare form of dysautonomia that causes fluctuations in your blood pressure^[10]. The baroreceptor reflex is one of your body’s automatic mechanisms that maintain your blood pressure, and BF is associated with the failure of part or all of this system. Symptoms include low resting blood pressure and severe hypertension (high blood pressure) when stressed.

Cerebral Salt Wasting Syndrome (CSWS)

CSWS is a rare condition that hyponatremia^[a] (low blood sodium) and persistent dehydration. It is usually a response to an injury to the brain or a result of tumors that are in or surrounding the brain. Low blood sodium is because, while the kidney functions regularly, it excretes excessive sodium^[11].

CSWS can often spontaneously resolve within a couple of months of appearing. However, it can occasionally last for months or even years. Symptoms are generally managed by increasing sodium and water intake to offset the excess losses. Additionally, certain medications can help increase blood sodium levels. 

These are only some forms of dysautonomia. In total, there are about 15 different identified conditions that fall under the dysautonomia umbrella. Understanding the various forms makes identifying which condition you may be experiencing easier, making the diagnosis process faster as your doctor knows what to screen for. 

Following a diagnosis of dysautonomia, it’s essential to devise a plan to manage symptoms. These conditions have no known cure, and while some may lie dormant or vanish over time (especially in the case of secondary dysautonomia, which can improve significantly once the primary condition is treated), other forms require significant lifestyle changes. A management program can substantially reduce the effect of certain symptoms and make it easier to resume your normal lifestyle.

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