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Pure Autonomic Failure

By Phillip Low, MD, Professor of Neurology; Consultant, Department of Neurology, College of Medicine, Mayo Clinic; Mayo Clinic

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Pure autonomic failure results from neuronal loss in autonomic ganglia, causing orthostatic hypotension and other autonomic symptoms.

Pure autonomic failure, previously called idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome, denotes generalized autonomic failure without CNS involvement. This disorder differs from multiple system atrophy because it lacks central or preganglionic involvement. Pure autonomic failure affects more women, tends to begin during a person’s 40s or 50s, and does not result in death.

Pure autonomic failure is a synucleinopathy (due to synuclein deposition); synuclein can also accumulate in patients with Parkinson disease, multiple system atrophy, or Lewy body dementia. (Synuclein is a neuronal and glial cell protein that can aggregate into insoluble fibrils and form Lewy bodies.) Some patients with pure autonomic failure eventually develop multiple system atrophy or Lewy body dementia.

Symptoms and Signs

The main symptom is

There may be other autonomic symptoms, such as decreased sweating, heat intolerance, urinary retention, bladder spasms (possibly causing incontinence), erectile dysfunction, fecal incontinence or constipation, and pupillary abnormalities.


  • Clinical evaluation

Diagnosis of pure autonomic failure is by exclusion. The norepinephrine level is usually < 100 pg/mL supine and does not increase with standing. Postural orthostatic tachycardia syndrome can be differentiated because with standing, it does not usually cause hypotension, the norepinephrine level increases, and heart rate increases by > 30 beats/min or to 120 beats/min within 10 min.


  • Symptomatic treatment

Treatment of pure autonomic failure is symptomatic:

  • Orthostatic hypotension: Volume expansion, vasopressors, and support hose

  • Constipation: High-fiber diet and stool softeners

  • Bladder spasms: Bladder antispasmodics

  • Urinary retention: Possibly self-catheterization of the bladder

  • Sweating abnormalities: Avoidance of hot conditions

Key Points

  • Pure autonomic failure, like Parkinson disease, multiple system atrophy, and Lewy body dementia, is a synucleinopathy.

  • The main symptom is orthostatic hypotension.

  • Diagnose by excluding other disorders that cause similar symptoms.

  • Use treatments specific for the symptoms present.