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Pure Autonomic Failure

By

Phillip Low

, MD, College of Medicine, Mayo Clinic

Last full review/revision Sep 2021| Content last modified Sep 2021
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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 Multiple System Atrophy (MSA) Multiple system atrophy is a relentlessly progressive neurodegenerative disorder causing pyramidal, cerebellar, and autonomic dysfunction. It includes 3 disorders previously thought to be distinct... read more 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.

Symptoms and Signs of Pure Autonomic Failure

The main symptom is

Diagnosis of Pure Autonomic Failure

  • 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 Postural orthostatic tachycardia syndrome (POTS) Orthostatic (postural) hypotension is an excessive fall in blood pressure (BP) when an upright position is assumed. The consensus definition is a drop of > 20 mm Hg systolic, 10 mm Hg diastolic... read more 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 of Pure Autonomic Failure

  • 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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