Bradbury-Eggleston syndrome

Bradbury-Eggleston Syndrome
Synonyms	Pure autonomic failure, Chronic idiopathic orthostatic hypotension
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Bradbury-Eggleston syndrome, also known as pure autonomic failure (PAF) or chronic idiopathic orthostatic hypotension, is a form of primary autonomic failure characterized by the degeneration of autonomic ganglia and the absence of other neurological abnormalities. This condition leads to significant orthostatic hypotension (a substantial decrease in blood pressure upon standing) without compensatory tachycardia (increased heart rate).

Symptoms and Signs

The hallmark of Bradbury-Eggleston syndrome is orthostatic hypotension, which can cause dizziness, lightheadedness, or fainting upon standing. Other symptoms may include:

• Fatigue
• Visual disturbances
• Neck pain
• Cognitive impairment, such as difficulty concentrating
• Syncope (fainting spells)
• Impotence in men

Causes

The exact cause of Bradbury-Eggleston syndrome is unknown. It is believed to result from the degeneration of postganglionic sympathetic neurons, leading to impaired autonomic regulation of blood pressure. The reason for this neuronal degeneration remains unclear, with no identifiable genetic, autoimmune, or infectious etiology.

Diagnosis

Diagnosis of Bradbury-Eggleston syndrome involves a clinical evaluation and several tests to assess autonomic function, including:

• Tilt table test
• Blood pressure measurements in different positions
• Heart rate variability tests
• Thermoregulatory sweat test
• Quantitative sudomotor axon reflex test (QSART)

Treatment

There is no cure for Bradbury-Eggleston syndrome, but treatment focuses on managing symptoms and improving quality of life. Treatment strategies may include:

• Increasing salt and fluid intake to raise blood volume
• Wearing compression garments to prevent blood from pooling in the legs
• Medications such as fludrocortisone to increase blood volume and midodrine to raise blood pressure
• Sleeping with the head elevated to reduce nocturnal hypertension and morning orthostatic hypotension

Prognosis

The prognosis for individuals with Bradbury-Eggleston syndrome varies. While the condition does not typically affect life expectancy, it can significantly impact quality of life. Management strategies can help control symptoms, but the condition may progressively worsen in some individuals.

See Also

• Orthostatic hypotension
• Autonomic nervous system
• Multiple system atrophy

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