Pure autonomic failure

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Pure Autonomic Failure

Pure Autonomic Failure (PAF), also known as Bradbury-Eggleston Syndrome or Idiopathic Orthostatic Hypotension, is a form of dysautonomia, a disorder of the autonomic nervous system (ANS). The pronunciation is /pjʊər ɔːˈtɒnəmɪk ˈfeɪljər/. The term was first coined in the 20th century, with 'autonomic' deriving from the Greek 'autonomos', meaning 'self-governing', and 'failure' from the Old French 'faillir', meaning 'to lack'.

Symptoms

The symptoms of PAF are related to the failure of the autonomic nervous system to regulate various body functions. These may include orthostatic hypotension, syncope (fainting), constipation, bladder dysfunction, and sexual dysfunction.

Causes

The exact cause of PAF is unknown, but it is thought to be due to degeneration of autonomic nerves in the brain and peripheral nervous system. It is not typically associated with other neurological disorders, distinguishing it from Multiple System Atrophy (MSA), which also involves autonomic failure but with additional neurological symptoms.

Diagnosis

Diagnosis of PAF is based on the presence of orthostatic hypotension without other neurological symptoms, and exclusion of other causes of autonomic failure. Tests may include tilt table test, autonomic function tests, and blood tests.

Treatment

Treatment of PAF is focused on managing symptoms, as there is currently no cure. This may involve medications to increase blood pressure, dietary changes, and physical measures such as compression stockings or elevating the head of the bed.

Prognosis

The prognosis for individuals with PAF varies. Some people may have mild symptoms and a normal lifespan, while others may experience significant disability and a reduced lifespan due to complications such as falls or cardiovascular disease.

See Also

External links

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