Pure autonomic failure
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Pure autonomic failure (PAF) is an uncommon, sporadic neurodegenerative condition marked by a steadily declining autonomic regulation.[3] Bradbury and Eggleston originally described pure autonomic failure in 1925.[4]
Pure autonomic failure | |
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Other names | Bradbury-Eggleston syndrome, idiopathic orthostatic hypotension.[1] |
Function of the autonomic nervous system. | |
Specialty | Neurology |
Symptoms | Orthostatic hypotension and other autonomic symptoms.[2] |
Diagnostic method | Clinical evaluation.[2] |
Treatment | Symptomatic treatment.[2] |
Patients usually present with orthostatic hypotension or syncope in midlife or later. In addition, genitourinary, thermoregulatory, and bowel dysfunction can be signs of autonomic failure.[5]
Pure autonomic failure originates from peripheral autonomic nervous system lesions.[6]
The diagnosis of pure autonomic failure relies on the absence of other neurologic abnormalities, specifically Parkinsonism, cognitive impairment, cerebellar ataxia, or tremors, and on compatible clinical features of subtle, progressive pan autonomic failure, most notably orthostatic hypotension.[7]