Autonomic Failure Syndrome (Pure Autonomic Failure)
- Degenerative disorder of the peripheral autonomic nervous system and considered to be one of the Lewy body diseases (1,2)
- Blood pressure (BP) drops with inadequate compensatory rise in heart rate while standing, such as symptomatic orthostatic hypotension, attributed to autonomic system impairment (1,3).
- Maintaining upright posture is difficult for patients with this disorder. They are at increased risk for fall and syncope.
Unconfirmed, area of ongoing research (4)
Etiology and Pathophysiology
- Actual cause not known; however, it impairs responses to homeostatic mechanism that regulate BP, causing loss of cerebral perfusion from poor arterial resistance, loss of increase in heart rate as compensation, and venous pooling in positional change (1).
- Neurodegenerative disease classified as an α-synucleinopathy, with Lewy bodies seen on histopathologic samples (1,2)
- No stimulation of sympathetic nervous system, and postganglionic sympathetic neurons do not release norepinephrine properly (1).
No specific genetic component identified
- Avoid abrupt change in posture and standing motionless for extended periods.
- Avoid lying completely supine if resting during day and elevate head of bed 6 to 9 inches at night (1).
- Remove any compressive stockings or abdominal bindings during supine positioning (1).
- Perform isometric or dynamic muscle contractions to squeeze venous blood upward to heart (1).
- Avoid large meals, hot environments (2).
- Drink adequate water (at least 2 to 2.5 L/day) and take salt at least 5 to 10 g/day (2).
Commonly Associated Conditions
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Domino, Frank J., et al., editors. "Autonomic Failure Syndrome (Pure Autonomic Failure)." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2020. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688699/all/Autonomic_Failure_Syndrome__Pure_Autonomic_Failure_.
Autonomic Failure Syndrome (Pure Autonomic Failure). In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters Kluwer; 2020. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688699/all/Autonomic_Failure_Syndrome__Pure_Autonomic_Failure_. Accessed May 28, 2023.
Autonomic Failure Syndrome (Pure Autonomic Failure). (2020). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5-Minute Clinical Consult (27th ed.). Wolters Kluwer. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688699/all/Autonomic_Failure_Syndrome__Pure_Autonomic_Failure_
Autonomic Failure Syndrome (Pure Autonomic Failure) [Internet]. In: Domino FJF, Baldor RAR, Golding JJ, Stephens MBM, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2020. [cited 2023 May 28]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688699/all/Autonomic_Failure_Syndrome__Pure_Autonomic_Failure_.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Autonomic Failure Syndrome (Pure Autonomic Failure) ID - 1688699 ED - Domino,Frank J, ED - Baldor,Robert A, ED - Golding,Jeremy, ED - Stephens,Mark B, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688699/all/Autonomic_Failure_Syndrome__Pure_Autonomic_Failure_ PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -