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Pure Autonomic Failure

By

Phillip Low

, MD, College of Medicine, Mayo Clinic

Last full review/revision Apr 2020| Content last modified Apr 2020
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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 central nervous system (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 dementia with Lewy bodies. (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 dementia with Lewy bodies.

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.

Diagnosis

  • 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/minutes or to 120 beats/minutes within 10 minutes.

Treatment

  • Symptomatic treatment

Treatment of pure autonomic failure is symptomatic:

Key Points

  • Pure autonomic failure, like Parkinson disease, multiple system atrophy, and dementia with Lewy bodies, 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.

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