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Neurogenic Orthostatic Hypotension
ADE WIJAYA, MD
Orthostatic Hypotension
A reduction in systolic blood pressure of at least 20 mm Hg or a
reduction in diastolic blood pressure of at least 10 mm Hg during the
first 3 minutes of standing or a head-up tilt on a tilt table
The definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. J Auton Nerv Syst 1996; 58:123-4
Orthostatic Hypotension Variant
Delayed orthostatic hypotension
• Occurs after 3 minutes of standing
• Represent mild or early form of symphatetic adrenergic dysfunction
Initial orthostatic hypotension
• Occurs within 15 seconds of standing
• Represent mismatch between cardiac output and peripheral
resistance
Gibbons CH, Freeman R. Delayed orthostatic hypotension: a frequent cause of orthostatic intolerance. Neurology 2006;67: 28-32.
Wieling W, Krediet CT, van Dijk N, 1. 2. 3. Linzer M, Tschakovsky ME. Initial orthostatic hypotension: review of a forgotten condition. Clin Sci (Lond) 2007;112:157- 65.
Epidemiology
More common in elderly due to:
o Reduced baroreflex responsiveness
o Decreased cardiac compliance
o Attenuation of the vestibulosympathetic reflex
Shibao C, Grijalva CG, Raj SR, Biaggioni I, Griffin MR. Orthostatic hypotension-related hospitalizations in the United States. Am J Med 2007;120:975-80
Freeman R. Neurogenic orthostatic hypotension. New England Journal of Medicine. 2008 Feb 7;358(6):615-24.
Clinical Features
Dizziness Light-headedness
Presyncope Syncope
Main Symptoms
In response to sudden postural change
Robertson D, Kincaid DW, Haile V, Robertson RM. The head and neck discomfort of autonomic failure: an unrecognized aetiology of headache. Clin Auton Res 1994;4:99-103
Additional Clinical Features
Generalized weakness
Fatigue
Nausea
Cognitive slowing
Leg buckling
Headache
Visual blurring
Neck pain
Orthostatic dyspnea / orthostatic angina
Robertson D, Kincaid DW, Haile V, Robertson RM. The head and neck discomfort of autonomic failure: an unrecognized aetiology of headache. Clin Auton Res 1994;4:99-103
Gibbons CH, Freeman R. Orthostatic. Orthostatic dyspnea: a neglected symptom of orthostatic hypotension. Clin Auton Res 2005; 15:40-4.
Shannon JR, Jordan J, Diedrich A, et al. Sympathetically mediated hypertension in autonomic failure. Circulation 2000;101: 2710-5
Neurogenic Orthostatic Hypotension
CENTRAL
Multiple-system atrophy (the Shy–Drager
syndrome)
Parkinson’s disease
Dementia with Lewy bodies
Pure autonomic failure
PERIPHERAL
Diabetes, amyloidosis, immune-mediated
neuropathies, hereditary sensory and
autonomic neuropathies (particularly
hereditary sensory and autonomic neuropathy
type III, also called familial dysautonomia), and
inflammatory neuropathies.
Less frequently: peripheral neuropathies that
accompany vitamin B12 deficiency, exposure
to neurotoxins, neuropathies due to infections,
including human immunodeficiency virus, and
porphyria
The definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. J Auton Nerv Syst 1996; 58:123-4.
Kaufmann H, Biaggioni I. Autonomic failure in neurodegenerative disorders. Semin Neurol 2003;23:351-63. Gilman S, Low PA, Quinn N, et al.
Consensus statement on the diagnosis of multiple system atrophy. J Auton Nerv Syst 1998;74:189-92
Low PA, Vernino S, Suarez G. Autonomic dysfunction in peripheral nerve disease. Muscle Nerve 2003;27:646-61.
Freeman R. Autonomic peripheral neuropathy. Lancet 2005;365:1259-70.
Evaluation
1. Dehydration and acute blood loss should be ruled out
2. Drugs (e.g., antihypertensive agents and antidepressants)
3. Reduced cardiac output (e.g., constrictive pericarditis, cardiomyopathy, and aortic stenosis)
4. Endocrine disorders (e.g., adrenal insufficiency and pheochromocytoma), and excessive
vasodilatation (e.g., systemic mastocytosis and the carcinoid syndrome)
5. Neurogenic orthostatic hypotension
Freeman R. Neurogenic orthostatic hypotension. New England Journal of Medicine. 2008 Feb 7;358(6):615-24.
Diagnostic Procedure
• Blood pressure should be measured while the patient is in the supine position and at least 3
minutes after the patient stands up
• Blood tests typically include a complete blood count, electrolyte assessment, blood glucose
level, serum immunoelectrophoresis, vitamin B12 level, and a morning cortisol level
• Brain MRI / SPECT
• Autonomic testing:
Bhattacharya K, Saadia D, Eisenkraft B, et al. Brain magnetic resonance imaging in multiple-system atrophy and Parkinson disease: a diagnostic algorithm. Arch Neurol 2002;59:835-42
Orimo S, Amino T, Uchihara T, et al. Decreased cardiac uptake of MIBG is a potential biomarker for the presence of Lewy bodies. J Neurol 2007;254:Suppl 4: IV21-IV28
Assessment: clinical autonomic testing report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 1996;46:873-80
Autonomic Testing
•The parasympathetic nervous system (e.g., heart rate variability with deep respiration and
during a Valsalva maneuver)
•The sympathetic cholinergic system (e.g., thermoregulatory sweat response and quantitative
sudomotor axon reflex test)
•The sympathetic adrenergic system (e.g., blood-pressure response to a Valsalva maneuver and a
tilt-table test with beat-to-beat measurement of blood pressure)
Assessment: clinical autonomic testing report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 1996;46:873-80
Treatment
Freeman R. Neurogenic orthostatic hypotension. New England Journal of Medicine. 2008 Feb 7;358(6):615-24.
Freeman R. Neurogenic orthostatic hypotension. New England Journal of Medicine. 2008 Feb 7;358(6):615-24.
Summary
A fall in systolic blood pressure of at least 20 mm Hg or in diastolic blood pressure of at least 10
mm Hg within 3 minutes after standing, is diagnostic of orthostatic hypotension
Non neurologic vs neurologic orthostatic hypotension
Reversible causes of orthostatic hypotension (in particular, the use of hypotensive medications)
should be addressed as soon as possible
Non pharmacological treatment vs pharmacological (fludrocortisone)
Neurogenic Orthostatic Hypotension

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Neurogenic Orthostatic Hypotension

  • 2. Orthostatic Hypotension A reduction in systolic blood pressure of at least 20 mm Hg or a reduction in diastolic blood pressure of at least 10 mm Hg during the first 3 minutes of standing or a head-up tilt on a tilt table The definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. J Auton Nerv Syst 1996; 58:123-4
  • 3. Orthostatic Hypotension Variant Delayed orthostatic hypotension • Occurs after 3 minutes of standing • Represent mild or early form of symphatetic adrenergic dysfunction Initial orthostatic hypotension • Occurs within 15 seconds of standing • Represent mismatch between cardiac output and peripheral resistance Gibbons CH, Freeman R. Delayed orthostatic hypotension: a frequent cause of orthostatic intolerance. Neurology 2006;67: 28-32. Wieling W, Krediet CT, van Dijk N, 1. 2. 3. Linzer M, Tschakovsky ME. Initial orthostatic hypotension: review of a forgotten condition. Clin Sci (Lond) 2007;112:157- 65.
  • 4. Epidemiology More common in elderly due to: o Reduced baroreflex responsiveness o Decreased cardiac compliance o Attenuation of the vestibulosympathetic reflex Shibao C, Grijalva CG, Raj SR, Biaggioni I, Griffin MR. Orthostatic hypotension-related hospitalizations in the United States. Am J Med 2007;120:975-80
  • 5. Freeman R. Neurogenic orthostatic hypotension. New England Journal of Medicine. 2008 Feb 7;358(6):615-24.
  • 6. Clinical Features Dizziness Light-headedness Presyncope Syncope Main Symptoms In response to sudden postural change Robertson D, Kincaid DW, Haile V, Robertson RM. The head and neck discomfort of autonomic failure: an unrecognized aetiology of headache. Clin Auton Res 1994;4:99-103
  • 7. Additional Clinical Features Generalized weakness Fatigue Nausea Cognitive slowing Leg buckling Headache Visual blurring Neck pain Orthostatic dyspnea / orthostatic angina Robertson D, Kincaid DW, Haile V, Robertson RM. The head and neck discomfort of autonomic failure: an unrecognized aetiology of headache. Clin Auton Res 1994;4:99-103 Gibbons CH, Freeman R. Orthostatic. Orthostatic dyspnea: a neglected symptom of orthostatic hypotension. Clin Auton Res 2005; 15:40-4. Shannon JR, Jordan J, Diedrich A, et al. Sympathetically mediated hypertension in autonomic failure. Circulation 2000;101: 2710-5
  • 8. Neurogenic Orthostatic Hypotension CENTRAL Multiple-system atrophy (the Shy–Drager syndrome) Parkinson’s disease Dementia with Lewy bodies Pure autonomic failure PERIPHERAL Diabetes, amyloidosis, immune-mediated neuropathies, hereditary sensory and autonomic neuropathies (particularly hereditary sensory and autonomic neuropathy type III, also called familial dysautonomia), and inflammatory neuropathies. Less frequently: peripheral neuropathies that accompany vitamin B12 deficiency, exposure to neurotoxins, neuropathies due to infections, including human immunodeficiency virus, and porphyria The definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. J Auton Nerv Syst 1996; 58:123-4. Kaufmann H, Biaggioni I. Autonomic failure in neurodegenerative disorders. Semin Neurol 2003;23:351-63. Gilman S, Low PA, Quinn N, et al. Consensus statement on the diagnosis of multiple system atrophy. J Auton Nerv Syst 1998;74:189-92 Low PA, Vernino S, Suarez G. Autonomic dysfunction in peripheral nerve disease. Muscle Nerve 2003;27:646-61. Freeman R. Autonomic peripheral neuropathy. Lancet 2005;365:1259-70.
  • 9. Evaluation 1. Dehydration and acute blood loss should be ruled out 2. Drugs (e.g., antihypertensive agents and antidepressants) 3. Reduced cardiac output (e.g., constrictive pericarditis, cardiomyopathy, and aortic stenosis) 4. Endocrine disorders (e.g., adrenal insufficiency and pheochromocytoma), and excessive vasodilatation (e.g., systemic mastocytosis and the carcinoid syndrome) 5. Neurogenic orthostatic hypotension Freeman R. Neurogenic orthostatic hypotension. New England Journal of Medicine. 2008 Feb 7;358(6):615-24.
  • 10. Diagnostic Procedure • Blood pressure should be measured while the patient is in the supine position and at least 3 minutes after the patient stands up • Blood tests typically include a complete blood count, electrolyte assessment, blood glucose level, serum immunoelectrophoresis, vitamin B12 level, and a morning cortisol level • Brain MRI / SPECT • Autonomic testing: Bhattacharya K, Saadia D, Eisenkraft B, et al. Brain magnetic resonance imaging in multiple-system atrophy and Parkinson disease: a diagnostic algorithm. Arch Neurol 2002;59:835-42 Orimo S, Amino T, Uchihara T, et al. Decreased cardiac uptake of MIBG is a potential biomarker for the presence of Lewy bodies. J Neurol 2007;254:Suppl 4: IV21-IV28 Assessment: clinical autonomic testing report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 1996;46:873-80
  • 11. Autonomic Testing •The parasympathetic nervous system (e.g., heart rate variability with deep respiration and during a Valsalva maneuver) •The sympathetic cholinergic system (e.g., thermoregulatory sweat response and quantitative sudomotor axon reflex test) •The sympathetic adrenergic system (e.g., blood-pressure response to a Valsalva maneuver and a tilt-table test with beat-to-beat measurement of blood pressure) Assessment: clinical autonomic testing report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 1996;46:873-80
  • 12. Treatment Freeman R. Neurogenic orthostatic hypotension. New England Journal of Medicine. 2008 Feb 7;358(6):615-24.
  • 13. Freeman R. Neurogenic orthostatic hypotension. New England Journal of Medicine. 2008 Feb 7;358(6):615-24.
  • 14. Summary A fall in systolic blood pressure of at least 20 mm Hg or in diastolic blood pressure of at least 10 mm Hg within 3 minutes after standing, is diagnostic of orthostatic hypotension Non neurologic vs neurologic orthostatic hypotension Reversible causes of orthostatic hypotension (in particular, the use of hypotensive medications) should be addressed as soon as possible Non pharmacological treatment vs pharmacological (fludrocortisone)