Table 2.

Major Causes of Autonomic Failure

Autonomic Synucleinopathies:

Multiple System Atrophy

A central nervous system cause of autonomic failure (with orthostatic hypotension) that may mimic some motor features of Parkinson’s Disease but is associated with a more rapid and dramatic course.

Parkinson’s Disease

A primary motor disease that can sometimes be associated with a peripheral autonomic neuropathy and orthostatic hypotension

Dementia with Lewy Bodies

A rapidly progressive dementia that is associated with a peripheral autonomic neuropathy and orthostatic hypotension.

Pure Autonomic Failure

A peripheral autonomic failure that occurs in older patients and can exist in isolation without motor neurological disorders.

Other Major Autonomic Disorders

Autoimmune Ganglionic Neuropathy

A form of autonomic failure caused by a loss of function antibody targeting of the alpha 3 subunit of the nicotinic receptor in autonomic ganglia.

Diabetic Autonomic Neuropathy

A peripheral autonomic neuropathy that can cause orthostatic hypotension, often in the setting of multiple other diabetic complications.

Familial Dysautonomia

An autosomal recessive developmental autonomic disease seen most commonly in Ashkenazi Jews.

Dopamine Beta Hydroxylase Deficiency

A rare genetic loss of the enzyme that is needed to convert dopamine to norepinephrine, so the patients lack both norepinephrine and epinephrine.

Amyloidosis

Amyloid protein deposition on autonomic neurons can cause autonomic failure with severe hypotension.

Drug-Induced Autonomic Dysfunction

Autonomic failure mimicked by drugs such as ganglionic blockers.

Spinal Cord Injury

Typically seen with cervical and thoracic spinal cord transections.