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Autonomic Failure Syndrome (Pure Autonomic Failure)

Zaiba Jetpuri, DO, MBA, CPH, FAAFP, Turya Nair, MD and Astrud San Antonio Villareal, MD Reviewed 06/2019
 


BASICS

DESCRIPTION

  • 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...

DIAGNOSIS

HISTORY

  • Dizziness or near syncope within seconds after standing, after a large meal (especially carbohydrate heavy), or in hot weather

  • Hypertension when lying flat

  • Neck pain in coat hanger dist...

TREATMENT

GENERAL MEASURES

  • Encourage physical exercise; avoid immobility.

  • Consider compression stockings, abdominal binder (1),(2)[C].

  • No specific BP goals (4)[C]

  • Avoid triggers for hypotension such as hi...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Work with primary care doctor to achieve symptom control and functional improvement. 

Patient Monitoring

Monitor for signs of weakness and light-headedness at home....

REFERENCES

1
Brown TP. Pure autonomic failure. Pract Neurol.  2017;17(5):341–348. [View Abstract on OvidMedline]
2
Thaisetthawatkul P. Pure autonomic failure. Curr Neurol Neurosci Rep.  2016;16...

CODES

ICD10

G90.8 Other disorders of autonomic nervous system 

ICD9

337.9 Unspecified disorder of autonomic nervous system 

SNOMED

  • 84438001 Pure autonomic failure

  • 15241006 Disorder of autonomic nervous syst...

CLINICAL PEARLS

  • Orthostatic hypotension defined by systolic BP drop of >20 mm Hg or diastolic BP drop >10 mm Hg within 3 minutes of standing or head-up tilt test of at least 60-degree angle (3)

  • P...

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