Pure autonomic failure
| Pure autonomic failure | |
|---|---|
| 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] |
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]
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]
- ^ "Monarch Initiative". Monarch Initiative. Retrieved February 2, 2024.
- ^ a b c Coon, Elizabeth (July 3, 2023). "Pure Autonomic Failure". Merck Manuals Professional Edition. Retrieved February 2, 2024.
- ^ Kabir, M. Ashish; Chelimsky, Thomas C. (2019). "Pure autonomic failure". Handbook of Clinical Neurology. Vol. 161. Elsevier. pp. 413–422. doi:10.1016/b978-0-444-64142-7.00064-3. ISBN 978-0-444-64142-7. ISSN 0072-9752.
- ^ Bradbury, Samuel; Eggleston, Cary (1925). "Postural hypotension". American Heart Journal. 1 (1). Elsevier BV: 73–86. doi:10.1016/s0002-8703(25)90007-5. ISSN 0002-8703.
- ^ Coon, Elizabeth A.; Singer, Wolfgang; Low, Phillip A. (2019). "Pure Autonomic Failure". Mayo Clinic Proceedings. 94 (10). Elsevier BV: 2087–2098. doi:10.1016/j.mayocp.2019.03.009. ISSN 0025-6196. PMC 6826339. PMID 31515103.
- ^ Garland, Emily M.; Hooper, William B.; Robertson, David (2013). "Pure autonomic failure". Autonomic Nervous System. Handbook of Clinical Neurology. Vol. 117. Elsevier. pp. 243–257. doi:10.1016/b978-0-444-53491-0.00020-1. ISBN 978-0-444-53491-0. ISSN 0072-9752. PMID 24095130.
- ^ Thaisetthawatkul, Pariwat (2016). "Pure Autonomic Failure". Current Neurology and Neuroscience Reports. 16 (8): 74. doi:10.1007/s11910-016-0673-2. ISSN 1528-4042. PMID 27338613.