Dysautonomia is an umbrella term to describe diseases of the autonomous nerve system.
The autonomous nerve system (vegetative nerve system) controls all involuntarily (not controllable) mechanisms of our body, such as heart rate and digestion.
We distinguish between primary and secondary dysautonomia. Primary dysautonomia occurs without an underlying cause; whereas secondary dysautonomia is a consequence of an underlying disease.
Dysfunction of the autonomous nerve system can affect every part of the body and may cause severe disability.
Types of Dysautnomia:
POTS (the most common type of Dysautonomia in EDS patients):
More information here.
Neurocardiogenic syncope (or neurally mediated syncope, neurally mediated hypotension): The most common type of dysautonomia. It describes a temporary loss of consciousness. It is probably caused by blood pooling in the legs while standing upright.
Multiple system atrophy: Similar to Parkinson’s this type of dysautonomia does also count to neurodegenerative diseases and is a primary dysautonomia.
Sinus tachycardia: High heart rate without physical activity and defined by a resting heart rate of 100 bpm.
Pure autonomic failure: PAF is a primary dysautonomia and a degenerative disease of peripheral nerve cells.
Autoimmune autonomic gangliopathy: AAG is an autoimmune disease where the body’s own immune system attacks autonomic ganglia.
Autonomic dysreflexia: AD has some connection to spinal cord injury.
Baroreflex failure: The baroreflex is a mechanism which stabilizes the blood pressure. In case of baroreflex failure this mechanism does not work correctly.
Diabetic autonomic neuropathy: A very common type of secondary dysautonomia due to diabetes.
Familial dysautonomia: This type of dysautonomia is very rare and genetic.
Reflex sympathetic dystrophy (complex regional pain syndrome, CRPS): This is more of a pain disorder but some doctors categorize it as a type of dysautonomia.
Orthostatic hypotension: Orthostatic hypotension describes a decrease of blood pressure when standing. The heart rate may rise to compensate the low blood pressure. OH is defined by a decrease of systolic blood pressure of 20 mmHg or diastolic blood pressure of 10 mmHg.
Depending on the type of dysautonomia the symptoms can affect every part of the body.
Possible symptoms may be:
Fatigue and exhaustion
Dizziness and vertigo
High heart rate
Low blood pressure
Bladder issues (irritable bladder, urinary retation, urinary urgency)
Strong sweating or no sweating at all
Heat and cold intolerance
Nausea and vomiting
Diabetes, MS, Parkinson’s, autoimmune diseases, alcohol abuse, medication, spinal cord injury, infections, connective tissue disorders.
ECG, Echo, blood tests (adrenaline, noradrenaline, infection, endocrinology), tilt table test with vasalva, Schellong test, neurophysiology, sweat test or QSART, stomach emptying test.
In patients with secondary dysautonomia the underlying cause is treated if possible.
In case of secondary dysautonomias where the underlying cause is not treatable or in case of primary dysautonomias a step wise approach is used.
This includes non-invasive treatment methods like higher salt intake, elevation of the head while sleeping, exercise of the leg muscles and cardiac exercises, compression tights, stockings and binder.
Medication that can be used are for example vasopressor, like Midodrine; medication that lead to an increase in blood volume, like fludrocortisone; betablocker and other blood pressure stabilizing medication.
Vagus Nerve Stimulation
As described in my article about pain management, there are studies going on right now on the effectiveness of vagus nerve stimulation in POTS patients. First results about other diseases such as Crohn’s disease or migraine look very promising. Since the vagus nerve plays an important role in the autonomic nervous system, it is plausible that stimulation could also have a positive effect on POTS or other dysautonomias.
Experimental and highly controversial:
New treatments of which you might have heard or eventually will hear, but which are NOT recommended by dysautonomia specialists at the moment nor FDA approved are:
Transvascular autonomic modulation (TVAM):
In case of transvascular autonomic modulation (TVAM) a balloon is inserted into a vein and inflated. The resulting reflex is supposed to improve sympathetic activity. This treatment is currently highly experimental and is heavily discussed, the opinions of dysautonomia specialists are currently rather negative, because there is a lack of scientific evidence and therefore the existing risk of this invasive procedure is not justified.
Another procedure that sometimes is used for dysautonomia patients with an autoimmune basis of their disease is the treatment with mesenchymal stem cells for which again there is not enough evidence at the moment.
Lee PH, Lee JE, Kim HS, Song SK, Lee HS, HS Nam Cheong JW, Jeong Y, Park HJ, Kim DJ, Nam CM. A randomized trial of mesenchymal stem cells in multiple system atrophy. Annals of neurology. 2012 Jul 1; 72 (1): 32-40.