Summary
Definition
History and exam
Key diagnostic factors
- presence of risk factors
- postural light-headedness, syncope, and other symptoms of cerebral hypoperfusion
Other diagnostic factors
- parkinsonian features
- cerebellar ataxia
- weight loss
- resting tachycardia or impaired heart rate variation
- abnormal gastrointestinal motility
- erectile dysfunction and lack of ejaculation
- anhidrosis, heat intolerance, dry skin, focal hyperhidrosis
- urinary frequency, urgency, nocturia
Risk factors
- older adult age
- frailty and physical deconditioning
- use of drugs that impair sympathetic tone
- volume depletion/anaemia
- autonomic neuropathy (e.g., diabetes mellitus)
- Parkinson's disease
- dementia with Lewy bodies
- multiple system atrophy
- hypertension
Diagnostic investigations
1st investigations to order
- posture test
Investigations to consider
- tilt-table test
- plasma noradrenaline (norepinephrine)
- deep breathing
- Valsalva manoeuvre
- nerve conduction studies and electromyogram (EMG)
- quantitative sudomotor axon reflex test (QSART)
- heart rate variability
- 24-hour blood pressure monitoring
- autoimmune antibodies
- chest CT
- serum and urine electrophoresis
- fat-pad biopsy
- genetic testing
Treatment algorithm
all patients
Contributors
Authors
Italo Biaggioni, MD
Professor of Medicine and Pharmacology
Vanderbilt University
Nashville
TN
Disclosures
IB is a consultant for Theravance Biopharma, Amneal Pharmaceuticals, Regeneron Pharmaceuticals, Takeda Pharmaceuticals, and Neurawell Therapeutics for development of therapies for orthostatic hypotension and postural tachycardia syndrome. He is also a patent holder for an automated abdominal binder for the treatment of orthostatic hypotension and has submitted a patent application for the use of guanfacine for the treatment of postural tachycardia and chronic fatigue syndromes.
Lucy Norcliffe-Kaufmann, PhD
Assistant Professor
Dysautonomia Center
Department of Physiology and Neuroscience
NYU Langone Medical Center
New York
NY
Disclosures
LNK is a board member of the American Autonomic Society and has received grant support from Theravance Biopharma, PTC Therapeutics, National Institutes of Health, Michael J. Fox Foundation, and the Familial Dysautonomia Foundation. LNK is an author of a number of references cited in this topic.
Horacio Kaufmann, MD
Professor of Neurology
Medicine and Pediatrics
New York University School of Medicine
New York
NY
Disclosures
HK is editor in chief of Clinical Autonomic Research and serves as principal investigator of a clinical trial sponsored by Biogen MA Inc. HK has received consultancy fees from Lilly USA LLC, Biohaven Pharmaceuticals Inc, Takeda Pharmaceutical Company Ltd, Ono Pharma UK Ltd, Lundbeck LLC, and Theravance Biopharma US Inc. HK is an author of a number of references cited in this topic.
Peer reviewers
Phillip A. Low, MD
Robert D. and Patricia E. Kern Professor of Neurology
Mayo Clinic College of Medicine
Rochester
MN
Disclosures
PAL is an author of a reference cited in this topic.
William P. Cheshire, MD
Professor of Neurology
Mayo Clinic
Jacksonville
FL
Disclosures
WPC declares that he has no competing interests.
Alan Moore, MB, FRCPI
Consultant Geriatrician
Beaumont Hospital
Dublin
Ireland
Disclosures
AM has received payment from Shire Pharmaceuticals, manufacturer of midodrine, for providing educational talks, and the department in which he works has received unrestricted educational grants from Shire Pharmaceuticals.
Differentials
- Neurally mediated (vasovagal) syncope
- Vertigo
- Non-specific falls in older people
More DifferentialsGuidelines
- Consensus statement on the definition of neurogenic supine hypertension
- Guidelines on the diagnosis and management of syncope
More GuidelinesPatient information
Postural hypotension (low blood pressure when you stand up)
More Patient information- Log in or subscribe to access all of BMJ Best Practice
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