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Pure Autonomic Failure

By

Phillip Low

, MD, College of Medicine, Mayo Clinic

Medically Reviewed Sep 2021 | Modified Sep 2022
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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... 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.

Drugs Mentioned In This Article

Drug Name Select Trade
Florinef
Orvaten, ProAmatine
Ditropan, Ditropan XL, Gelnique , Oxytrol, Oxytrol for Women
Detrol, Detrol LA
Myrbetriq
Duvoid , Urecholine, Urecholine Solution
Advanced Eye Relief, Akwa Tears, Akwa Tears Renewed, Alcon Tears, Artificial Tears, Bion Tears, Blink Tears, Clear eyes, Clear eyes Advance Dry and Itchy Relief, Clear eyes Outdoor Dry Eye Protection, Eye Lubricant , FreshKote, Gen Teal Moderate to Severe, GenTeal , GenTeal Mild, GenTeal Moderate, GenTeal PF, GenTeal Severe, GenTeal Tears Mild, GenTeal Tears Severe Dry Eye, Gonak, Goniosoft, Hypo Tears , Isopto Tears, LiquiTears, LubriFresh P.M., Moisture Eyes, Moisture Eyes Preservative Free, Murine, Natural Balance Tears, Nature's Tears, Opti-Free, Puralube Tears, Refresh, Refresh Celluvisc, Refresh Endura, Refresh Lacri-Lube, Refresh Liquigel, Systane, Systane Balance, Systane Complete, Systane Ultra, Teargen, Tears Naturale Forte, Tears Naturale Free, Tears Naturale II, Tears Renewed , TheraTears, Visine Dry Eye Relief, Visine Maximum Redness Relief, Visine Pure Tears, Visine Tears, Visine Tired Eye Relief, Viva
Revatio, Viagra
Adcirca, ALYQ, Cialis, Tadliq
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