(See also Overview of the Autonomic Nervous System.)
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
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
Treatment of pure autonomic failure is symptomatic:
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Orthostatic hypotension: Volume expansion, vasopressors, and support hose
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Constipation: High-fiber diet and stool softeners
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Bladder spasms: Bladder antispasmodics
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Urinary retention: Possibly self-catheterization of the bladder
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Sweating abnormalities: Avoidance of hot conditions
