History

Fact Explanation
A known predisposing medical syndrome Some syndromes are associated with chronic orthostatic hypotension, together with their respective characteristic features. i.e. Diabetes mellitus (due to autonomic neuropathy) [1] [2] [3] [4] [5], idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome (due to idiopathic degeneration of the peripheral autonomic nerves)[1] [6] [7], familial dysautonomia (autosomal recessive condition) [1] [8] [9] [10] [11], baroreceptor dysfunction [12] due to neck irradiation, trauma, surgery, oropharyngeal carcinoma [1]) and rarely, dopamine-beta-hydroxylase deficiency (which causes a severe orthostatic hypotension [13] [14] [15] ) Some medical conditions cause peripheral neuropathy and thus causes orthostatic hypotension. i.e. diabetes mellitus, alcoholism, nutritional deficiencies (vitamin B12, folic acid), Guillain-barre syndrome, toxins, drugs, heavy metals, hereditary neuropathies, Hansen's disease, paraneoplastic syndrome, renal failure, recurrent peripheral neuropathy, amyloidosis, porphyria, infectious agents (diphtheria, tetanus, botulism), systemic collagen vascular disease, syringomyelia and Tabes dorsalis. [1]
A known predisposing drug The common drugs that cause orthostatic hypotension are nitrates, vasodilators, alcohol, narcotics, tricyclic antidepressants, prolonged use of sympathomimetics and phenothiazines. [1] [16] [17] [18]
Characteristic clinical symptoms on standing up Independent of the cause, almost all the patients feel dizziness, faintishness, lightheadedness or swaying when they stand up or sit up after recumbency. [1] In some patients it may be short-lived [19], in some, comes suddenly and disappears after a short while [20] [21] [22], but in some case, may be even a chronic experience. [7] Most of the time, the symptoms are relieved by recumbency, or kept standing up for a few minutes. [19] The cause is cerebral hypotension [23] due to failure of upright reflex. [19] In patients with autonomic failure orthostatic hypotension results from an impaired capacity to increase vascular resistance during standing. [24] The sequence of this is increased downward pooling of venous blood and a consequent reduction in stroke volume and cardiac output that exaggerates the orthostatic fall in blood pressure. [24] [25] [26] [27] [28] [29]
Fits Prolonged cerebral ischemia in orthostatic intolerence can cause focal cerebral necrosis and seizures. [1] [30] [31] They can be any type, tonic-clonic or aphasic. [1]
Sudden onset weakness, facial deviation Features of stroke, which is possible following severe and long standing ischemia to the brain. [1] [31]
Headache An atypical manifestation, could be misdiagnosed, and is due to cerebral ischemia. [1] [32] [33]
Neck pain, backache Atypical presentation, and due to muscle hypoperfusion. [1] [34]
Chest pain Due to compensatory autonomic over-reaction. [1] [34] [35]
References
  1. HOLLISTER AS. Orthostatic hypotension. Causes, evaluation, and management. West J Med [online] 1992 Dec, 157(6):652-657 [viewed 28 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1022100
  2. HILSTED J, PARVING HH, CHRISTENSEN NJ, BENN J, GALBO H. Hemodynamics in diabetic orthostatic hypotension. J Clin Invest [online] 1981 Dec, 68(6):1427-1434 [viewed 28 June 2014] Available from: doi:10.1172/JCI110394
  3. LAEDERACH-HOFMANN KURT, WEIDMANN PETER, FERRARI PAOLO. Hypovolemia contributes to the pathogenesis of orthostatic hypotension in patients with diabetes mellitus. The American Journal of Medicine [online] 1999 January, 106(1):50-58 [viewed 28 June 2014] Available from: doi:10.1016/S0002-9343(98)00367-2
  4. EGUCHI K, PICKERING T G, ISHIKAWA J, HOSHIDE S, KOMORI T, TOMIZAWA H, SHIMADA K, KARIO K. Severe orthostatic hypotension with diabetic autonomic neuropathy successfully treated with a β1-blocker: a case report. J Hum Hypertens [online] December, 20(10):801-803 [viewed 28 June 2014] Available from: doi:10.1038/sj.jhh.1002066
  5. LANIER JB, MOTE MB and CLAY EC. Evaluation and Management of Orthostatic Hypotension. Am Fam Physician. [online] 2011 Sep 1;84(5):527-536. [viewed 29 June 2014] Available at http://www.aafp.org/afp/2011/0901/p527.html
  6. IDIáQUEZ J, KAUFMANN H, SOZA M, NECOCHEA C. [Pure autonomic failure. Bradbury Eggleston Syndrome. Case report]. Rev Med Chil [online] 2005 Feb, 133(2):215-8 [viewed 28 June 2014] Available from: doi:/S0034-98872005000200010
  7. ROBERTSON DAVID. Causes of Chronic Orthostatic Hypotension. Arch Intern Med [online] 1994 July [viewed 28 June 2014] Available from: doi:10.1001/archinte.1994.00420140086011
  8. SHOHAT M, and HALPREN GJ. GeneReviews®: Familial Dysautonomia [web] [viewed 28 June 2014] Available at http://www.ncbi.nlm.nih.gov/books/NBK1180/
  9. GOLD-VON SIMSON GABRIELLE, GOLDBERG JUDITH D, ROLNITZKY LINDA M, MULL JAMES, LEYNE MAIRE, VOUSTIANIOUK ANDREI, SLAUGENHAUPT SUSAN A, AXELROD FELICIA B. Kinetin in Familial Dysautonomia Carriers: Implications for a New Therapeutic Strategy Targeting mRNA Splicing. Pediatr Res [online] 2009 March, 65(3):341-346 [viewed 28 June 2014] Available from: doi:10.1203/PDR.0b013e318194fd52
  10. AXELROD FB. Familial dysautonomia. Muscle Nerve [online] 2004 Mar, 29(3):352-63 [viewed 28 June 2014] Available from: doi:10.1002/mus.10499
  11. AXELROD FB. Familial dysautonomia: a review of the current pharmacological treatments. Expert Opin Pharmacother [online] 2005 Apr, 6(4):561-7 [viewed 28 June 2014] Available from: doi:10.1517/14656566.6.4.561
  12. BRIASOULIS ALEXANDROS, SILVER ADAM, YANO YUICHIRO, BAKRIS GEORGE L.. Orthostatic Hypotension Associated With Baroreceptor Dysfunction: Treatment Approaches. J Clin Hypertens [online] December, 16(2):141-148 [viewed 28 June 2014] Available from: doi:10.1111/jch.12258
  13. ROBERTSON D, GARLAND EM, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Dopamine Beta-Hydroxylase Deficiency [online] 1993 [viewed 28 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301647
  14. ROBERTSON D, HAILE V, PERRY SE, ROBERTSON RM, PHILLIPS JA 3RD, BIAGGIONI I. Dopamine beta-hydroxylase deficiency. A genetic disorder of cardiovascular regulation. Hypertension [online] 1991 Jul, 18(1):1-8 [viewed 28 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/1677640
  15. TIMMERS HJ, DEINUM J, WEVERS RA, LENDERS JW. Congenital dopamine-beta-hydroxylase deficiency in humans. Ann N Y Acad Sci [online] 2004 Jun:520-3 [viewed 28 June 2014] Available from: doi:10.1196/annals.1296.064
  16. NOURIAN Z., MOW T., MUFTIC D., BUREK S., PEDERSEN M. L., MATZ J., MULVANY M. J.. Orthostatic hypotensive effect of antipsychotic drugs in Wistar rats by in vivo and in vitro studies of α1-adrenoceptor function. Psychopharmacology [online] December, 199(1):15-27 [viewed 29 June 2014] Available from: doi:10.1007/s00213-007-1064-9
  17. ROBERTSON DAVID. Causes of Chronic Orthostatic Hypotension. Arch Intern Med [online] 1994 July [viewed 29 June 2014] Available from: doi:10.1001/archinte.1994.00420140086011
  18. GUPTA VISHAL, LIPSITZ LEWIS A.. Orthostatic Hypotension in the Elderly: Diagnosis and Treatment. The American Journal of Medicine [online] 2007 October, 120(10):841-847 [viewed 29 June 2014] Available from: doi:10.1016/j.amjmed.2007.02.023
  19. STEWART JULIAN M., CLARKE DEBBIE. “He’s Dizzy When He Stands Up”: An Introduction to Initial Orthostatic Hypotension. The Journal of Pediatrics [online] 2011 March, 158(3):499-504 [viewed 29 June 2014] Available from: doi:10.1016/j.jpeds.2010.09.004
  20. WEISS AVRAHAM, BELOOSESKY YICHAYAOU, KORNOWSKI RAN, YALOV ALEXANDRA, GRINBLAT JOSEPH, GROSSMAN EHUD. Influence of orthostatic hypotension on mortality among patients discharged from an acute geriatric ward. J Gen Intern Med [online] 2006 June, 21(6):602-606 [viewed 29 June 2014] Available from: doi:10.1111/j.1525-1497.2006.00450.x
  21. KUJAWA KATHY, LEURGANS SUE, RAMAN REMA, BLASUCCI LUCY, GOETZ CHRISTOPHER G.. Acute Orthostatic Hypotension When Starting Dopamine Agonists in Parkinson's Disease. Arch Neurol [online] 2000 October [viewed 29 June 2014] Available from: doi:10.1001/archneur.57.10.1461
  22. WEISS AVRAHAM, GROSSMAN EHUD, BELOOSESKY YICHAYAOU, GRINBLAT JOSEPH. Orthostatic Hypotension in Acute Geriatric Ward. Arch Intern Med [online] 2002 November [viewed 29 June 2014] Available from: doi:10.1001/archinte.162.20.2369
  23. WIELING WOUTER, KREDIET C. T. PAUL, VAN DIJK NYNKE, LINZER MARK, TSCHAKOVSKY MICHAEL E.. Initial orthostatic hypotension: review of a forgotten condition. Clinical Science [online] 2007 February [viewed 29 June 2014] Available from: doi:10.1042/CS20060091
  24. SMIT AA, HALLIWILL JR, LOW PA, WIELING W. Pathophysiological basis of orthostatic hypotension in autonomic failure. J Physiol [online] 1999 Aug 15:1-10 [viewed 29 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/10432334
  25. METZLER MANUELA, DUERR SUSANNE, GRANATA ROBERTA, KRISMER FLORIAN, ROBERTSON DAVID, WENNING GREGOR K.. Neurogenic orthostatic hypotension: pathophysiology, evaluation, and management. J Neurol [online] December, 260(9):2212-2219 [viewed 29 June 2014] Available from: doi:10.1007/s00415-012-6736-7
  26. MEDOW MARVIN S., STEWART JULIAN M., SANYAL SANJUKTA, MUMTAZ ARIF, SICA DOMENIC, FRISHMAN WILLIAM H.. Pathophysiology, Diagnosis, and Treatment of Orthostatic Hypotension and Vasovagal Syncope. Cardiology in Review [online] 2008 January, 16(1):4-20 [viewed 29 June 2014] Available from: doi:10.1097/CRD.0b013e31815c8032
  27. MAULE SIMONA, PAPOTTI GRAZIA, NASO DIEGO, MAGNINO CORRADO, TESTA ELISA, VEGLIO FRANCO. Orthostatic hypotension: evaluation and treatment. CHDDT [online] 2007 March, 7(1):63-70 [viewed 29 June 2014] Available from: doi:10.2174/187152907780059029
  28. TYKOCKI T, GUZEK K, NAUMAN P. [Orthostatic hypotension and supine hypertension in primary autonomic failure. Pathophysiology, diagnosis and treatment]. Kardiol Pol [online] 2010 Sep, 68(9):1057-63 [viewed 29 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20859904
  29. MANO T., IWASE S.. Sympathetic nerve activity in hypotension and orthostatic intolerance. Acta Physiol Scand [online] 2003 March, 177(3):359-365 [viewed 29 June 2014] Available from: doi:10.1046/j.1365-201X.2003.01081.x
  30. HIERONS R, SHELDON J. Attacks of Orthostatic Hypotension Resembling Focal Epilepsy Associated with Diabetes and Hypoparathyroidism Proc R Soc Med [online] 1956 Jun, 49(6):333-334 [viewed 29 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1888913
  31. RILEY TERRENCE L.. Stroke, Orthostatic Hypotension, and Focal Seizures. JAMA [online] 1981 March [viewed 29 June 2014] Available from: doi:10.1001/jama.1981.03310370035020
  32. ARBOGAST STEVEN D., ALSHEKHLEE AMER, HUSSAIN ZULFIQAR, MCNEELEY KEVIN, CHELIMSKY THOMAS C.. Hypotension Unawareness in Profound Orthostatic Hypotension. The American Journal of Medicine [online] 2009 June, 122(6):574-580 [viewed 29 June 2014] Available from: doi:10.1016/j.amjmed.2008.10.040
  33. KURAMAE T, INAMASU J, NAKAGAWA Y, NAKATSUKASA M. Spontaneous intracranial hypotension presenting without orthostatic headache complicated by acute subdural hematoma after drainage for chronic subdural hematoma--case report. Neurol Med Chir (Tokyo) [online] 2011, 51(7):518-21 [viewed 29 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21785248
  34. MATHIAS C. J., MALLIPEDDI RAJEEV, BLEASDALE-BARR KATHARINE. Symptoms associated with orthostatic hypotension in pure autonomic failure and multiple system atrophy. [online] 1999 October, 246(10):893-898 [viewed 29 June 2014] Available from: doi:10.1007/s004150050479
  35. FIGUEROA J. J., BASFORD J. R., LOW P. A.. Preventing and treating orthostatic hypotension: As easy as A, B, C. Cleveland Clinic Journal of Medicine [online] December, 77(5):298-306 [viewed 29 June 2014] Available from: doi:10.3949/ccjm.77a.09118

Examination

Fact Explanation
Low blood pressure with a postural drop The blood pressure while recumbent and after standing up/sitting up should be measured. In one study the majority reached their maximum systolic decrease within 5 minutes of standing, but in 20-30% the maximum blood pressure drop occurred after 5 minutes or later, in 38%, the systolic blood pressure drop was more than 40 mm Hg. [1] But according to the 1996 consensus definition, orthostatic hypotension is diagnosed when a fall in systolic blood pressure of at least 20 mm Hg and/or diastolic blood pressure of at least 10 mm Hg within 3 min of standing is recorded. [2] [3] [4]
Normal or mildly elevated pulse rate The reason behind reduced cerebral perfusion is lack of normal sympathetic reflexive response to suddenly low blood pressure. Unlike in syncopal attacks, the pulse rate is either normal or mildly elevated if any, and it also can be irregular. [2] [5] [6] [7] [8] [9]
Anhydrosis Because of dysautonomia. [2] [10] [11] [12] Amount of sweating in the dorsum of the foot is a tool used to assess autonomic dysfunction. [13]
Bladder distension A feature of dysautonomia and neurogenic orthostatic hypotension. Bladder stasis comes earlier than symptomatic hypotension. One must be aware to ask about erectile dysfunction in such patients. [14] [15] [16] [17] [18] [19] [20] [21]
Motor weakness There is a significant reduction of blood flow in brain in patients with orthostatic hypotension, which is a potent risk factor for cerebral ischemia and stroke syndromes later. [22] [23] [24] [25]
Sensory deficit Due to cerebral ischemia. [26]
Cerebellar signs Due to ischaemia in cerebellum. Commonly seen in multiple system atrophy. [27] [28] [29]
A seizure Due to cerebral ischemia. [25] [30]
References
  1. PASSANT U, WARKENTIN S, GUSTAFSON L. Orthostatic hypotension and low blood pressure in organic dementia: a study of prevalence and related clinical characteristics. Int J Geriatr Psychiatry [online] 1997 Mar, 12(3):395-403 [viewed 29 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/9152727, DOI: 10.1002/(SICI)1099-1166(199703)12:3<395::AID-GPS527>3.0.CO;2-#
  2. NASCHITZ JE, ROSNER I. Orthostatic hypotension: framework of the syndrome Postgrad Med J [online] 2007 Sep, 83(983):568-574 [viewed 29 June 2014] Available from: doi:10.1136/pgmj.2007.058198
  3. KREDIET C. T. PAUL, GO-SCHöN INGEBORG K., KIM YU-SOK, LINZER MARK, VAN LIESHOUT JOHANNES J., WIELING WOUTER. Management of initial orthostatic hypotension: lower body muscle tensing attenuates the transient arterial blood pressure decrease upon standing from squatting. Clinical Science [online] 2007 November [viewed 29 June 2014] Available from: doi:10.1042/CS20070064
  4. VASUDEV AKSHYA, O'BRIEN JOHN T., TAN MAW PIN, PARRY STEVE W., THOMAS ALAN J.. A study of orthostatic hypotension, heart rate variability and baroreflex sensitivity in late-life depression. Journal of Affective Disorders [online] 2011 June, 131(1-3):374-378 [viewed 01 July 2014] Available from: doi:10.1016/j.jad.2010.11.001
  5. STEWART JM, CLARKE D. "He's Dizzy when he Stands Up." An Introduction to Initial orthostatic Hypotension J Pediatr [online] 2011 Mar, 158(3):499-504 [viewed 01 July 2014] Available from: doi:10.1016/j.jpeds.2010.09.004
  6. AUNG AK, CORCORAN SJ, NAGALINGAM V, PAUL E, NEWNHAM HH. Prevalence, Associations, and Risk Factors for Orthostatic Hypotension in Medical, Surgical, and Trauma Inpatients: An Observational Cohort Study Ochsner J [online] 2012, 12(1):35-41 [viewed 01 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3307503
  7. WARD CHRISTOPHER, KENNY ROSE ANNE. Reproducibility of orthostatic hypotension in symptomatic elderly. The American Journal of Medicine [online] 1996 April, 100(4):418-422 [viewed 01 July 2014] Available from: doi:10.1016/S0002-9343(97)89517-4
  8. SHI X, WRAY DW, FORMES KJ, WANG HW, HAYES PM, O-YURVATI AH, WEISS MS, REESE IP. Orthostatic hypotension in aging humans. Am J Physiol Heart Circ Physiol [online] 2000 Oct, 279(4):H1548-54 [viewed 01 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/11009440
  9. AGARWAL A K, GARG R, RITCH A, SARKAR P. Postural orthostatic tachycardia syndrome. Postgraduate Medical Journal [online] 2007 July, 83(981):478-480 [viewed 01 July 2014] Available from: doi:10.1136/pgmj.2006.055046
  10. MATHIAS C. J., MALLIPEDDI RAJEEV, BLEASDALE-BARR KATHARINE. Symptoms associated with orthostatic hypotension in pure autonomic failure and multiple system atrophy. [online] 1999 October, 246(10):893-898 [viewed 01 July 2014] Available from: doi:10.1007/s004150050479
  11. REICHGOTT MJ. Clinical Methods: The History, Physical, and Laboratory Examinations. 3rd edition; Chapter 76: Clinical Evidence of Dysautonomia. [web] [viewed 1 July 2014] Available on http://www.ncbi.nlm.nih.gov/books/NBK400/
  12. NANDA RN, JOHNSON RH. Orthostatic hypotension associated with paroxysmal ventricular tachycardia. J Neurol Neurosurg Psychiatry [online] 1975 Jul, 38(7):698-702 [viewed 01 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1083250
  13. ITOH H, UEBORI S, ASAI M, KASHIWAYA T, ATOH K, MAKINO I. Early detection of orthostatic hypotension by quantitative sudomotor axon reflex test (QSART) in type 2 diabetic patients. Intern Med [online] 2003 Jul, 42(7):560-4 [viewed 01 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/12879946
  14. SAKAKIBARA R, HATTORI T, UCHIYAMA T, KITA K, ASAHINA M, SUZUKI A, YAMANISHI T. Urinary dysfunction and orthostatic hypotension in multiple system atrophy: which is the more common and earlier manifestation? J Neurol Neurosurg Psychiatry [online] 2000 Jan, 68(1):65-69 [viewed 01 July 2014] Available from: doi:10.1136/jnnp.68.1.65
  15. METZLER MANUELA, DUERR SUSANNE, GRANATA ROBERTA, KRISMER FLORIAN, ROBERTSON DAVID, WENNING GREGOR K.. Neurogenic orthostatic hypotension: pathophysiology, evaluation, and management. J Neurol [online] December, 260(9):2212-2219 [viewed 01 July 2014] Available from: doi:10.1007/s00415-012-6736-7
  16. SCHROEDER CHRISTOPH, JORDAN JENS, KAUFMANN HORACIO. Management of Neurogenic Orthostatic Hypotension in Patients with Autonomic Failure. Drugs [online] December, 73(12):1267-1279 [viewed 01 July 2014] Available from: doi:10.1007/s40265-013-0097-0
  17. TYKOCKI T, GUZEK K, NAUMAN P. [Orthostatic hypotension and supine hypertension in primary autonomic failure. Pathophysiology, diagnosis and treatment]. Kardiol Pol [online] 2010 Sep, 68(9):1057-63 [viewed 01 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20859904
  18. MAULE SIMONA, PAPOTTI GRAZIA, NASO DIEGO, MAGNINO CORRADO, TESTA ELISA, VEGLIO FRANCO. Orthostatic Hypotension: Evaluation and Treatment. CHDDT [online] 2007 March, 7(1):63-70 [viewed 01 July 2014] Available from: doi:10.2174/187152907780059029
  19. PAPATSORIS A.G., PAPAPETROPOULOS S., SINGER C., DELIVELIOTIS C.. Urinary and erectile dysfunction in multiple system atrophy (MSA). Neurourol. Urodyn. [online] December, 27(1):22-27 [viewed 01 July 2014] Available from: doi:10.1002/nau.20461
  20. KIRCHHOF K, APOSTOLIDIS AN, MATHIAS CJ, FOWLER CJ. Erectile and urinary dysfunction may be the presenting features in patients with multiple system atrophy: a retrospective study. Int J Impot Res [online] 2003 Aug, 15(4):293-8 [viewed 01 July 2014] Available from: doi:10.1038/sj.ijir.3901014
  21. VINIK AI, MASER RE, MITCHELL BD, FREEMAN R. Diabetic autonomic neuropathy. Diabetes Care [online] 2003 May, 26(5):1553-79 [viewed 01 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/12716821
  22. TREGER IULY, SHAFIR OLEG, KEREN OFER, RING HAIM. Orthostatic hypotension and cerebral blood flow velocity in the rehabilitation of stroke patients. International Journal of Rehabilitation Research [online] 2006 December, 29(4):339-342 [viewed 01 July 2014] Available from: doi:10.1097/MRR.0b013e328010c87d
  23. EIGENBRODT M. L., ROSE K. M., COUPER D. J., ARNETT D. K., SMITH R., JONES D.. Orthostatic Hypotension as a Risk Factor for Stroke : The Atherosclerosis Risk in Communities (ARIC) Study, 1987-1996. Stroke [online] 2000 October, 31(10):2307-2313 [viewed 01 July 2014] Available from: doi:10.1161/​01.STR.31.10.2307
  24. KONG KENG-HE, CHUO ADELENE M.. Incidence and outcome of orthostatic hypotension in stroke patients undergoing rehabilitation. Archives of Physical Medicine and Rehabilitation [online] 2003 April, 84(4):559-562 [viewed 01 July 2014] Available from: doi:10.1053/apmr.2003.50040
  25. RILEY TL, FRIEDMAN JM. Stroke, orthostatic hypotension, and focal seizures. JAMA [online] 1981 Mar 27, 245(12):1243-4 [viewed 01 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/6782252
  26. CLAYDON VICTORIA E., KRASSIOUKOV ANDREI V.. Orthostatic Hypotension and Autonomic Pathways after Spinal Cord Injury. Journal of Neurotrauma [online] 2006 December, 23(12):1713-1725 [viewed 01 July 2014] Available from: doi:10.1089/neu.2006.23.1713
  27. WENNING GREGOR KARL, GRANATA ROBERTA, KRISMER FLORIAN, DüRR SUSANNE, SEPPI KLAUS, POEWE WERNER, BLEASDALE-BARR KATHARINE, MATHIAS CHRISTOPHER J.. Orthostatic Hypotension Is Differentially Associated with the Cerebellar Versus the Parkinsonian Variant of Multiple System Atrophy: a Comparative Study. Cerebellum [online] December, 11(1):223-226 [viewed 01 July 2014] Available from: doi:10.1007/s12311-011-0299-5
  28. HUGHES RC, CARTLIDGE NE, MILLAC P. Primary neurogenic orthostatic hypotension J Neurol Neurosurg Psychiatry [online] 1970 Jun, 33(3):363-371 [viewed 01 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC493480
  29. KöLLENSPERGER MARTIN, GESER FELIX, SEPPI KLAUS, STAMPFER-KOUNTCHEV MICHAELA, SAWIRES MARTIN, SCHERFLER CHRISTOPH, BOESCH SYLVIA, MUELLER JOERG, KOUKOUNI VASILIKI, QUINN NIALL, PELLECCHIA MARIA TERESA, BARONE PAOLO, SCHIMKE NICOLE, DODEL RICHARD, OERTEL WOLFGANG, DUPONT ERIK, ØSTERGAARD KAREN, DANIELS CHRISTINE, DEUSCHL GüNTHER, GUREVICH TANYA, GILADI NIR, COELHO MIGUEL, SAMPAIO CRISTINA, NILSSON CHRISTER, WIDNER HåKAN, SORBO FRANCESCA DEL, ALBANESE ALBERTO, CARDOZO ADRIANA, TOLOSA EDUARDO, ABELE MICHAEL, KLOCKGETHER THOMAS, KAMM CHRISTOPH, GASSER THOMAS, DJALDETTI RUTH, COLOSIMO CARLO, MECO GIUSEPPE, SCHRAG ANETTE, POEWE WERNER, WENNING GREGOR K.. Red flags for multiple system atrophy. Mov Disord. [online] 2008 June, 23(8):1093-1099 [viewed 01 July 2014] Available from: doi:10.1002/mds.21992
  30. HIERONS R, SHELDON J. Attacks of Orthostatic Hypotension Resembling Focal Epilepsy Associated with Diabetes and Hypoparathyroidism Proc R Soc Med [online] 1956 Jun, 49(6):333-334 [viewed 01 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1888913

Differential Diagnoses

Fact Explanation
Syncopal attacks Syncopal attacks usually have the same characteristic features. The predisposing factors, aura and the transient loss of consciousness with rapid recovery with no or minimal residual symptoms are a scope of it. The commonest is vasovagal syncope. Examination findings are low systolic blood pressure and tachycardia, with pallor and clamminess. [1] [2] [3] [4] The causative factors might be evident in the history. [5] Severe orthostatic hypotension might be associated with recurrent syncopal attacks as well. [6]
Hypoglycemia Sine a considerable percentage of diabetic patients present with autonomic neuropathy, it's always important to check an emergency setting blood glucose (capillary blood glucose) to exclude hypoglycemia. [7] [8] [9]
References
  1. BRIGNOLE M. Diagnosis and treatment of syncope Heart [online] 2007 Jan, 93(1):130-136 [viewed 05 July 2014] Available from: doi:10.1136/hrt.2005.080713
  2. LEMPERT T. Recognizing syncope: pitfalls and surprises. J R Soc Med [online] 1996 Jul, 89(7):372-375 [viewed 05 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1295849
  3. MILLER TH and KRUSE JE. Evaluation of Syncope. Am Fam Physician. [online] 2005 Oct 15;72(8):1492-1500 [viewed 05 July 2014] Available from http://www.aafp.org/afp/2005/1015/p1492.html
  4. CHEN-SCARABELLI C, SCARABELLI TM. Neurocardiogenic syncope BMJ [online] 2004 Aug 7, 329(7461):336-341 [viewed 05 July 2014] Available from: doi:10.1136/bmj.329.7461.336
  5. HOLLISTER AS. Orthostatic hypotension. Causes, evaluation, and management. West J Med [online] 1992 Dec, 157(6):652-657 [viewed 05 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1022100
  6. KAWAKAMI KAZUNOBU, ABE HARUHIKO, HARAYAMA NOBUYA, NAKASHIMA YASUHIDE. Successful Treatment of Severe Orthostatic Hypotension with Erythropoietin. Pacing and Clinical Electrophysiology [online] 2003 January, 26(1p1):105-107 [viewed 05 July 2014] Available from: doi:10.1046/j.1460-9592.2003.00158.x
  7. SARVGHADI F and YASARI F. Causes and Clinical Presentation of Hypoglycemia in Patients with Low Blood Glucose Admitted to the Emergency Ward. International Journal of Endocrinology and Metabolism. [online] 2007 Dec 5 (4) Available from http://endometabol.com/?page=article&article_id=1982
  8. SHAFIEE GITA, MOHAJERI-TEHRANI MOHAMMADREZA, PAJOUHI MOHAMMAD, LARIJANI BAGHER. The importance of hypoglycemia in diabetic patients. Array [online] 2012 December [viewed 05 July 2014] Available from: doi:10.1186/2251-6581-11-17
  9. CRYER PE, DAVIS SN, SHAMOON H. Hypoglycemia in diabetes. Diabetes Care [online] 2003 Jun, 26(6):1902-12 [viewed 05 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/12766131

Investigations - for Diagnosis

Fact Explanation
Investigate for the cause Since there are one too many causes for orthostatic hypotension, specific investigations for the etiological diagnosis should be carried out. i. e. neurodegenerative diseases, parkisonism, amyloidosis, diabetes, anemia, and vitamin deficiency. The type of investigation should be decided by the the attending doctor. [1]
Head upright tilt-test This examines the baroreceptor-reflex system as a whole entity in a non-invasive way. Staying upright requires rapid circulatory and neurologic compensation as soon as the posture changes, to maintain blood pressure and consciousness. If one of these are ineffective or if there is insufficient blood volume, the location of the brain above the heart and the presence of large venous reservoirs below the heart causes cardiac filling and blood pressure to decrease rapidly with consequent cerebral mal-perfusion and loss of consciousness. The mechanism behind the head tiling test is such, but the severity of orthostatic intolerance cannot be predicted by the positivity of the test. Most of the patients do become positive with three minutes of upright posture. [2] [3] [4] [5] [6] [7]
Valsalva manoeuvre In the patients with orthostatic intolerence in three minutes, most had failed valsalva maneouvre especially stage two and four. [2] [8]
References
  1. GUPTA VISHAL, LIPSITZ LEWIS A.. Orthostatic Hypotension in the Elderly: Diagnosis and Treatment. The American Journal of Medicine [online] 2007 October, 120(10):841-847 [viewed 06 July 2014] Available from: doi:10.1016/j.amjmed.2007.02.023
  2. GIBBONS CH, FREEMAN R. Delayed orthostatic hypotension: a frequent cause of orthostatic intolerance. Neurology [online] 2006 Jul 11, 67(1):28-32 [viewed 11 July 2014] Available from: doi:10.1212/01.wnl.0000223828.28215.0b
  3. STEWART JULIAN M., CLARKE DEBBIE. “He’s Dizzy When He Stands Up”: An Introduction to Initial Orthostatic Hypotension. The Journal of Pediatrics [online] 2011 March, 158(3):499-504 [viewed 11 July 2014] Available from: doi:10.1016/j.jpeds.2010.09.004
  4. GEHRKING JADE A., HINES STACY M., BENRUD-LARSON LISA M., OPHER-GEHRKING TONETTE L., LOW PHILLIP A.. What is the minimum duration of head-up tilt necessary to detect orthostatic hypotension?. Clin Auton Res [online] 2005 April, 15(2):71-75 [viewed 11 July 2014] Available from: doi:10.1007/s10286-005-0246-y
  5. NASCHITZ J E. The patient with supine hypertension and orthostatic hypotension: a clinical dilemma. Postgraduate Medical Journal [online] 2006 April, 82(966):246-253 [viewed 11 July 2014] Available from: doi:10.1136/pgmj.2005.037457
  6. National Guideline Clearinghouse. Ortgostatic hypotension. [web] [viewed 11 July 2014] Available at http://www.guideline.gov/content.aspx?id=34904
  7. SCHUTZMAN JOHN, JAEGER FREDRICK, MALONEY JAMES, FOUAD-TARAZI FETNAT. Head-up tilt and hemodynamic changes during orthostatic hypotension in patients with supine hypertension. Journal of the American College of Cardiology [online] 1994 August, 24(2):454-461 [viewed 11 July 2014] Available from: doi:10.1016/0735-1097(94)90303-4
  8. HUGHES RC, CARTLIDGE NE, MILLAC P. Primary neurogenic orthostatic hypotension J Neurol Neurosurg Psychiatry [online] 1970 Jun, 33(3):363-371 [viewed 11 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC493480

Investigations - Fitness for Management

Fact Explanation
12 lead Electrocardiogram To assess cardiac fitness of the patient and exclude other cardiovascular co-morbidities.
References
  1. WU J.-S., YANG Y.-C., LU F.-H., WU C.-H., WANG R.-H., CHANG C.-J.. Population-Based Study on the Prevalence and Risk Factors of Orthostatic Hypotension in Subjects With Pre-Diabetes and Diabetes. Diabetes Care [online] 2009 January, 32(1):69-74 [viewed 11 July 2014] Available from: doi:10.2337/dc08-1389

Management - General Measures

Fact Explanation
Compression garments i.e. Abdominal compression. Usefull in cases where there is adrenergic denervation of vascular beds, in those cases, there is an increase in vascular capacitance and peripheral venous pooling. Compression of capacitance beds (i.e, the legs and abdomen) improves orthostatic symptoms. The improvement is due to a reduction of venous capacitance and an increase in total peripheral resistance. Compressing legs is not as effective as compressing the abdomen. [1] [2] [3] [4] [5] Once combined together with other postural maneuvers accelerates the recovery. [6]
Postural adjustment Raising the head-end of the bed when patient is supine also reduced the drop of blood pressure thus symptoms when standing. During the day, adequate orthostatic stress, i.e, upright activity, should be maintained. If patients are repeatedly tilted up, their orthostatic hypotension is gradually attenuated, presumably by increasing venomotor tone. [1] [7] Combination with other treatment options yields better results. [6]
Counter-maneuvers Physical countermaneuvers are isometrical contracting of the muscles below the waist for about 30 seconds at a time, which reduces venous capacitance, increases total peripheral resistance, and augments venous return to the heart. These countermeasures can help maintain blood pressure during daily activities and should be considered at the first symptoms of orthostatic intolerance and in situations of orthostatic stress. Some of the known specific techniques include toe-raising, leg-crossing and contraction, thigh muscle co-contraction, bending at the waist, slow marching in place and leg elevation.
Education of the patient and the family It's the cornerstone of the management. Education about orthostatic stressors and warning symptoms empowers the patient to adopt easy lifestyle changes to minimize and handle orthostatic stress. [1] [9] [10]
References
  1. FIGUEROA JJ, BASFORD JR, LOW PA. Preventing and treating orthostatic hypotension: As easy as A, B, C Cleve Clin J Med [online] 2010 May, 77(5):298-306 [viewed 06 July 2014] Available from: doi:10.3949/ccjm.77a.09118
  2. SMIT ADRIANUSA.J., WIELING WOUTER, FUJIMURA JIRO, DENQ JONGC., OPFER-GEHRKING TONETTEL., AKARRIOU MOHAMMED, KAREMAKER JOHNM., LOW PHILLIPA.. Use of lower abdominal compression to combat orthostatic hypotension in patients with autonomic dysfunction. Clin Auton Res [online] 2004 June [viewed 06 July 2014] Available from: doi:10.1007/s10286-004-0187-x
  3. TUTAJ M., MARTHOL H., BERLIN D., BROWN C. M., AXELROD F. B., HILZ M. J.. Effect of physical countermaneuvers on orthostatic hypotension in familial dysautonomia. J Neurol [online] December, 253(1):65-72 [viewed 06 July 2014] Available from: doi:10.1007/s00415-005-0928-3
  4. ROWELL LB, DETRY JM, BLACKMON JR, WYSS C. Importance of the splanchnic vascular bed in human blood pressure regulation. J Appl Physiol [online] 1972 Feb, 32(2):213-20 [viewed 06 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/4550275
  5. SJOSTRAND T. Volume and distribution of blood and their significance in regulating the circulation. Physiol Rev [online] 1953 Apr, 33(2):202-28 [viewed 06 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/13055444
  6. HILZ M. J., EHMANN E. C., PAULI E., BALTADZHIEVA R., KOEHN J., MOELLER S., DEFINA P., AXELROD F. B.. Combined counter-maneuvers accelerate recovery from orthostatic hypotension in familial dysautonomia. Acta Neurol Scand [online] December, 126(3):162-170 [viewed 06 July 2014] Available from: doi:10.1111/j.1600-0404.2012.01670.x
  7. CHANDLER MARGARET P., MATHIAS CHRISTOPHER J.. Haemodynamic responses during head-up tilt and tilt reversal in two groups with chronic autonomic failure: pure autonomic failure and multiple system atrophy. [online] 2002 May, 249(5):542-548 [viewed 06 July 2014] Available from: doi:10.1007/s004150200062
  8. ASAHINA MASATO, YOUNG TIM M., BLEASDALE-BARR KATHARINE, MATHIAS CHRISTOPHER J.. Differences in overshoot of blood pressure after head-up tilt in two groups with chronic autonomic failure: pure autonomic failure and multiple system atrophy. J Neurol [online] 2005 January, 252(1):72-77 [viewed 06 July 2014] Available from: doi:10.1007/s00415-005-0609-2
  9. FREEMAN R. Treatment of Orthostatic Hypotension. Semin Neurol [online] 2003 December, 23(4):435-442 [viewed 06 July 2014] Available from: doi:10.1055/s-2004-817727
  10. FIGUEROA J. J., BASFORD J. R., LOW P. A.. Preventing and treating orthostatic hypotension: As easy as A, B, C. Cleveland Clinic Journal of Medicine [online] December, 77(5):298-306 [viewed 06 July 2014] Available from: doi:10.3949/ccjm.77a.09118

Management - Specific Treatments

Fact Explanation
Alpha-stimulating vasopressors Midodrine is the commonly used vasopressive agent, being an alpha-adrenergic stimulant, raises arterial blood pressure independent of the route of administration. It is effective and safe when used for treating neurogenic orthostatic hypotension. It has been shown to increase standing systolic blood pressure, reduce orthostatic light-headedness, and increase standing and walking time. The common starting dose is 5 mg three times a day; most patients respond best to 10 mg three times a day. As its duration of action is short (2 to 4 hours), it should be taken before arising in the morning, before lunch, and in the midafternoon. To avoid nocturnal supine hypertension, doses should not be taken after the midafternoon, and a dose should be omitted if the supine or sitting blood pressure is greater than 180/100 mm Hg. The patient should be educated about the side effects of the drug, being supine hypertension, scalp paresthesias, and pilomotor reactions (goosebumps). [1] [2] [3] [4] [5] [6] [7]
Volume expansors Fludrocortisone is the commonest used volume expansor. It's a synthetic mineralocorticoid with negligible glucocorticoid action. The action is pressor effect as a result of its ability to expand plasma volume and increase vascular alpha-adrenoceptor sensitivity. This drug is helpful when plasma volume fails to adequately increase with salt supplementation, and for patients who cannot ingest enough salt or do not respond adequately to midodrine. The usual dose is 0.1 to 0.2 mg/day, but it may be increased to 0.4 to 0.6 mg/day in patients with refractory orthostatic hypotension. If the patient gains 1.2–2.3 kg and develops mild dependent edema, it's inferable that the plasma volume has expanded adequately. Fludrocortisone is contraindicated in congestive heart failure and chronic renal failure. The potential risks are severe hypokalemia and excessive supine hypertension, and the patient shoud be eductaed about these effects. Frequent monitoring of serum potassium, a diet high in potassium, and regular checks of supine blood pressure are advised, especially at higher doses, when added to midodrine, or in elderly patients who tend to poorly tolerate the medication. [1] [5] [8] [9] [10] [11]
Choline-esterase inhibitors It improves neurotransmission at acetylcholine-mediated neuronal reflexes of the autonomic nervous system. Because this pathway is activated primarily during standing, this drug improves orthostatic hypotension and total peripheral resistance without aggravating supine hypertension. Since the pressor effect is modest, it is most adequate for patients with mild to moderate orthostatic hypotension. Dosing is started at 30 mg two to three times a day and is gradually increased to 60 mg three times a day. The drug’s effectiveness can be enhanced by combining each dose of pyridostigmine with 5 mg of midodrine without occurrence of supine hypertension. The patient shoud be eductaed to look out for adverse effects of the drug i.e. abdominal colic, diarrhea. [1] [5] [12] [13] [14] [15] [16] [17]
References
  1. FIGUEROA JJ, BASFORD JR, LOW PA. Preventing and treating orthostatic hypotension: As easy as A, B, C Cleve Clin J Med [online] 2010 May, 77(5):298-306 [viewed 06 July 2014] Available from: doi:10.3949/ccjm.77a.09118
  2. CRUZ DINNA N. Midodrine: a selective α-adrenergic agonist for orthostatic hypotension and dialysis hypotension. Expert Opin. Pharmacother. [online] 2000 May, 1(4):835-840 [viewed 06 July 2014] Available from: doi:10.1517/14656566.1.4.835
  3. JANKOVIC JOSEPH, GILDEN JANICE L., HINER BRADLEY C., KAUFMANN HORACIO, BROWN DAVID C., COGHLAN CECIL H., RUBIN MICHAEL, FOUAD-TARAZI FETNAT M.. Neurogenic orthostatic hypotension: A double-blind, placebo-controlled study with midodrine. The American Journal of Medicine [online] 1993 July, 95(1):38-48 [viewed 06 July 2014] Available from: doi:10.1016/0002-9343(93)90230-M
  4. HOELDTKE ROBERT D., HORVATH GABRIELLA G., BRYNER KIMBERLY D., HOBBS GERALD R.. Treatment of Orthostatic Hypotension with Midodrine and Octreotide . The Journal of Clinical Endocrinology & Metabolism [online] 1998 February, 83(2):339-343 [viewed 06 July 2014] Available from: doi:10.1210/jcem.83.2.4534
  5. MAULE SIMONA, PAPOTTI GRAZIA, NASO DIEGO, MAGNINO CORRADO, TESTA ELISA, VEGLIO FRANCO. Cardiovascular & Hematological Disorders-Drug Targets. CHDDT [online] 2007 March, 7(1):63-70 [viewed 06 July 2014] Available from: doi:10.2174/187152907780059029
  6. LOW PHILLIP A.. Efficacy of Midodrine vs Placebo in Neurogenic Orthostatic Hypotension. JAMA [online] 1997 April [viewed 06 July 2014] Available from: doi:10.1001/jama.1997.03540370036033
  7. FOUAD-TARAZI FETNAT M., OKABE MASANORI, GOREN HERSHEL. Alpha sympathomimetic treatment of autonomic insufficiency with orthostatic hypotension. The American Journal of Medicine [online] 1995 December, 99(6):604-610 [viewed 06 July 2014] Available from: doi:10.1016/S0002-9343(99)80246-0
  8. HUSSAIN RM, MCINTOSH SJ, LAWSON J, KENNY RA. Fludrocortisone in the treatment of hypotensive disorders in the elderly. Heart [online] 1996 Dec, 76(6):507-509 [viewed 06 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC484603
  9. SCHOFFER KERRIE L., HENDERSON ROBERT D., O'MALEY KAREN, O'SULLIVAN JOHN D.. Nonpharmacological treatment, fludrocortisone, and domperidone for orthostatic hypotension in Parkinson's disease. Mov Disord. [online] December, 22(11):1543-1549 [viewed 06 July 2014] Available from: doi:10.1002/mds.21428
  10. CHOBANIAN ARAM V., VOLICER LADISLAV, TIFFT CHARLES P., GAVRAS HARALAMBOS, LIANG CHANG-SENG, FAXON DAVID. Mineralocorticoid-Induced Hypertension in Patients with Orthostatic Hypotension. N Engl J Med [online] 1979 July, 301(2):68-73 [viewed 06 July 2014] Available from: doi:10.1056/NEJM197907123010202
  11. AXELROD FELICIA B., GOLDBERG JUDITH D., ROLNITZKY LINDA, MULL JAMES, MANN SANDRA P., GOLD VON SIMSON GABRIELLE, BERLIN DENA, SLAUGENHAUPT SUSAN A.. Fludrocortisone in patients with familial dysautonomia. Clin Auton Res [online] 2005 August, 15(4):284-291 [viewed 06 July 2014] Available from: doi:10.1007/s10286-005-0288-1
  12. LANIER JB, MOTE MB and CLAY EC. Evaluation and Management of Orthostatic Hypotension. Am Fam Physician. [online] 2011 Sep 1;84(5):527-536. [viewed 06 July 2014] Available from: http://www.aafp.org/afp/2011/0901/p527.html
  13. SINGER WOLFGANG, SANDRONI PAOLA, OPFER-GEHRKING TONETTE L., SUAREZ GUILLERMO A., KLEIN CAROLINE M., HINES STACY, O’BRIEN PETER C., SLEZAK JEFFREY, LOW PHILLIP A.. Pyridostigmine Treatment Trial in Neurogenic Orthostatic Hypotension. Arch Neurol [online] 2006 April [viewed 06 July 2014] Available from: doi:10.1001/archneur.63.4.noc50340
  14. SINGER W. Acetylcholinesterase inhibition: a novel approach in the treatment of neurogenic orthostatic hypotension. Journal of Neurology, Neurosurgery & Psychiatry [online] 2003 September, 74(9):1294-1298 [viewed 06 July 2014] Available from: doi:10.1136/jnnp.74.9.1294
  15. LOW PA, SINGER W. Update on Management of Neurogenic Orthostatic Hypotension Lancet Neurol [online] 2008 May, 7(5):451-458 [viewed 06 July 2014] Available from: doi:10.1016/S1474-4422(08)70088-7
  16. SHIBAO CYNDYA, LIPSITZ LEWIS ARNOLD, BIAGGIONI ITALO. ASH Position Paper: Evaluation and Treatment of Orthostatic Hypotension. [online] December, 15(3):147-153 [viewed 06 July 2014] Available from: doi:10.1111/jch.12062
  17. SINGER WOLFGANG, SANDRONI PAOLA, OPFER-GEHRKING TONETTE L., SUAREZ GUILLERMO A., KLEIN CAROLINE M., HINES STACY, O’BRIEN PETER C., SLEZAK JEFFREY, LOW PHILLIP A.. Pyridostigmine Treatment Trial in Neurogenic Orthostatic Hypotension. Arch Neurol [online] 2006 April [viewed 06 July 2014] Available from: doi:10.1001/archneur.63.4.noc50340