Rare Dysautonomias

In addition to more commonly diagnosed dysautonomias, such as POTS or neurocardiogenic syncope, there are a number of less-known dysautonomias that are seen far less often. They include:

Multiple System Atrophy (MSA) is a progressive disease that breaks down multiple portions of the central and autonomic nervous systems.

This condition causes symptoms similar to those seen in Parkinson’s disease, including widespread nerve damage and failure of digestive, urinary, speech, swallowing and cardiovascular functions.

MSA can affect anyone, but is most common in men over 50. The cause of MSA is unknown at this time.


Also known as idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome, Pure Autonomic Failure (PAF) is marked by persistent drops in blood pressure upon standing, WITHOUT any signs of central nervous system dysfunction or any known cause of orthostatic hypotension (OH). The cause of the drop in blood pressure is failure of the sympathetic neurocirculatory system.

PAF patients are rare, and experience more severe OH and increasingly worsening dizziness when standing up, as well as after eating a large amount of food, heat exposure or exercise. Researchers believe the cause of PAF is the loss of sympathetic nerve function.

PAF usually occurs during the middle age or elderly years, with no greater incidence with any sex or race. It can evolve into Parkinson’s disease with OH or Lewdy body dementia (the second-most-common form of dementia).


A genetic disorder that occurs primarily in people of Eastern European Jewish descent. Both the autonomic and sensory nervous systems are affected.


A condition that results from damage to nerves that control parts of the autonomic nervous system due to the effects of diabetes. Nerve damage can include those controlling cardiovascular, gastrointestinal, urinary, endocrine and reproductive functions. Neurogenic orthostatic hypotension may also occur if the sympathetic noradrenergic system’s baroreflex regulation fails.


Caused by injury high in the spinal cord, this condition causes dangerously high blood pressure in response to the filling of the bladder. This action causes a reflexive signal to be sent to the cardiovascular system, and when the baroreflex does not work correctly, blood pressure is affected in the extreme.


With this form of dysautonomia, the brain doesn’t respond correctly to signals from the cardiovascular system, which prompts the sympathetic noradrenergic system to respond inappropriately. This can result in extremely high or low blood pressure and heart rate. Baroreflexes cannot keep these functions in check, which prompts extreme reactions from relatively minor stimuli, such as sitting up from a lying down position.

Information on this page comes from Principles of Autonomic Medicine by Dr. David Goldstein, MD PhD



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