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.

Etiology is usually unknown. Some cases are due to a synucleinopathy (see Movement and Cerebellar Disorders: Pathophysiology); occasionally, the cause is an autoimmune autonomic neuropathy.

The main symptom is orthostatic hypotension; 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 is by exclusion. The norepinephrineSome Trade Names
LEVOPHED
Click for Drug Monograph
level is usually < 100 pg/mL supine and does not increase with standing. Postural tachycardia syndrome can be differentiated because with standing, it does not usually cause hypotension, the norepinephrineSome Trade Names
LEVOPHED
Click for Drug Monograph
level increases, and heart rate increases by > 30 beats/min or to 120 beats/min within 10 min.

Treatment

Treatment is symptomatic:

• Orthostatic hypotension: 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

Last full review/revision November 2009 by Phillip Low, MD

Content last modified February 2012