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Pure Autonomic failure

– by Dr. Ratna Bhavaraju-Sanka
 

Other names: Idiopathic orthostatic hypotension, Bradbury-Eggleston Syndrome, Neurogenic orthostatic hypotension

 

Cardinal Features:

• Idiopathic orthostatic hypotension (Drop in BP by 20 mm Hg systolic or 10 mm diastolic) within 3 minutes of assuming upright position. If the patient has history of hypertension, orthostatic hypotension is defined as a drop in BP with upright position by 30 mm Hg systolic
• No history of Parkinsonism
• May have problems with bladder and sexual function
• Commonly seen in mid-late adulthood
• Synucleopathy with synuclein deposits in lewy bodies found in autonomic ganglia
 

Symptoms:

• Dizziness/ unsteadiness or near fainting on standing up from sitting or lying down position
• Symptoms worse in morning, or after meals or after exercise/physical activity
• reduced sweating, heat intolerance
• urinary problems like hesitancy, urgency, incontinence, urinary retention and frequent infections
• Sexual dysfunction like impotence, erectile and ejaculatory problems
 

Signs:

• Fall in blood pressure by >20 mm systolic or 10 mm diastolic after at least 1 minute of standing.
• Abnormal autonomic testing showing autonomic insufficiency.
• low supine norepinephrine levels with no or minimal increase on standing up.
• Unremarkable brain imaging.
• Neurological exam with no signs of movement disorders
 

Differential diagnosis:

• Multiple system atrophy
• Parkinson’s disease with orthostatic hypotension
 

Prognosis:

It is a chronic illness with slow progression. It can also have episodes of worsening due to problems like dehydration, intercurrent illness (infections), medications, deconditioning.
 
It is important to differentiate this from Multiple system atrophy and Parkinson’s disease with orthostatic hypotension. Pure autonomic failure is less progressive and generally is not as disabling as the other two mentioned above.

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