Research Articles Library

This library is made up of many reseach articles written on dysautonomia and its related conditions. It discusses examinations of the causes of these conditions, symptoms, and treatments. We hope that this resource is useful to patients and providers alike.

Table of Contents:

 
​General Dysautonomia Journal Articles

​2015 Heart Rhythm Society Expert Consensus Statement on the Diagnosis and Treatment of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia, and Vasovagal Syncope

  • Sheldon RS, Grubb BP, Olshansky B, et al. 2015 Heart Rhythm Society Expert Consensus Statement on the Diagnosis and Treatment of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia, and Vasovagal Syncope. Heart rhythm. 2015;12(6):e41-e63. doi:10.1016/j.hrthm.2015.03.029.

 

Orthostatic Changes in Hemodynamics and Cardiovascular Biomarkers in Dysautonomic Patients

  • Nilsson D, Sutton R, Tas W, Burri P, Melander O, Fedorowski A. Orthostatic Changes in Hemodynamics and Cardiovascular Biomarkers in Dysautonomic Patients. Björklund P, ed. PLoS ONE. 2015;10(6):e0128962. doi:10.1371/journal.pone.0128962.

Management of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia and Vasovagal Syncope

  • Raj S, Sheldon R. Management of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia and Vasovagal Syncope. Arrhythmia & Electrophysiology Review. 2016;5(2):122-129. doi:10.15420/AER.2016.7.2.

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POTS Journal Articles

Good Overview of POTS

Postural Tachycardia Syndrome: Beyond Orthostatic Intolerance

  • Garland EM, Celedonio JE, Raj SR. Postural Tachycardia Syndrome: Beyond Orthostatic Intolerance. Current neurology and neuroscience reports. 2015;15(9):60. doi:10.1007/s11910-015-0583-8. 

POTS Diagnosis

Diagnosing POTS, Tilt Table vs. Standing Hemodynamic PDF

  • Plash WB, Diedrich A, Biaggioni I, Garland EM, Paranjape SY, Black BK, Dupont WD, Raj SR. Diagnosing postural tachycardia syndrome: comparison of tilt testing compared with standing haemodynamics. Clin Sci (Lond). 2013 Jan;124(2):109-14. PubMed PMID: 22931296; PubMed Central PMCID: PMC3478101.

Unspecified POTS

Diurnal Variability in POTS PDF

  • Brewster JA, Garland EM, Biaggioni I, Black BK, Ling JF, Shibao CA, Robertson D, Raj SR. Diurnal variability in orthostatic tachycardia: implications for the postural tachycardia syndrome. Clin Sci (Lond). 2012 Jan;122(1):25-31. doi: 10.1042/CS20110077. PubMed PMID: 21751966; PubMed Central PMCID: PMC3172399.

Autoimmune Basis for POTS PDF

  • Li H, Yu X, Liles C, Khan M, Vanderlinde-Wood M, Galloway A, Zillner C, Benbrook A, Reim S, Collier D, Hill MA, Raj SR, Okamoto LE, Cunningham MW, Aston CE, Kem DC. Autoimmune basis for postural tachycardia syndrome. J Am Heart Assoc. 2014 Feb 26;3(1):e000755. doi: 10.1161/JAHA.113.000755. PubMed PMID: 24572257; PubMed Central PMCID: PMC3959717.

Brain Fog in POTS PDF

  • Ross AJ, Medow MS, Rowe PC, Stewart JM. What is brain fog? An evaluation of the symptom in postural tachycardia syndrome. Clin Auton Res. 2013 Dec;23(6):305-11. doi: 10.1007/s10286-013-0212-z. Epub 2013 Sep 3. PubMed PMID: 23999934; PubMed Central PMCID: PMC3896080.

A Prospective, 1-year Follow Up Study of POTS PDF

  • Kimpinski K, Figueroa JJ, Singer W, et al. A Prospective, 1-Year Follow-up Study of Postural Tachycardia Syndrome. Mayo Clinic Proceedings. 2012;87(8):746-752. doi:10.1016/j.mayocp.2012.02.020.

Some CFS Patients Have POTS PDF

  • Lewis I, Pairman J, Spickett G, Newton JL. Clinical characteristics of a novel subgroup of chronic fatigue syndrome patients with postural orthostatic tachycardia syndrome. J Intern Med. 2013 May;273(5):501-10. doi: 10.1111/joim.12022. Epub 2013 Jan 7. PubMed PMID: 23206180.

Pregnancy/Gynecology in POTS

Gynecological Disorders and Menstrual Cycle Lightheadedness in POTS PDF

  • Peggs KJ, Nguyen H, Enayat D, Keller NR, Al-Hendy A, Raj SR. Gynecologic disorders and menstrual cycle lightheadedness in postural tachycardia syndrome. Int J Gynaecol Obstet. 2012 Sep;118(3):242-6. doi: 10.1016/j.ijgo.2012.04.014. Epub 2012 Jun 20. PubMed PMID: 22721633; PubMed Central PMCID: PMC3413773.

Menstrual Cycle Affects POTS PDF

  • Fu Q, VanGundy TB, Shibata S, Auchus RJ, Williams GH, Levine BD. Menstrual cycle affects renal-adrenal and hemodynamic responses during prolonged standing in the postural orthostatic tachycardia syndrome. Hypertension. 2010 Jul;56(1):82-90. doi: 10.1161/HYPERTENSIONAHA.110.151787. Epub 2010 May 17. PubMed PMID: 20479333; PubMed Central PMCID: PMC2894615.

Pregnancy in POTS: Maternal & Fetal Outcomes PDF 

  • Kimpinski K, Iodice V, Sandroni P, Low PA. Effect of pregnancy on postural tachycardia syndrome. Mayo Clin Proc. 2010 Jul;85(7):639-44. doi: 10.4065/mcp.2009.0672. Epub 2010 Jun 1. PubMed PMID: 20516426; PubMed Central PMCID: PMC2894719

Outcomes of Pregnancy in POTS

  • Kanjwal K, Karabin B, Kanjwal Y, Grubb BP. Outcomes of pregnancy in patients with preexisting postural tachycardia syndrome. Pacing Clin Electrophysiol. 2009 Aug;32(8):1000-3. doi: 10.1111/j.1540-8159.2009.02430.x. PubMed PMID: 19659618.

Anxiety/Mental Health in POTS

Excessive Heart Rate in POTS Not Due to Anxiety PDF

  • Masuki S, Eisenach JH, Johnson CP, Dietz NM, Benrud-Larson LM, Schrage WG, Curry TB, Sandroni P, Low PA, Joyner MJ. Excessive heart rate response to orthostatic stress in postural tachycardia syndrome is not caused by anxiety. J Appl Physiol (1985). 2007 Mar;102(3):896-903. Epub 2006 Nov 16. PubMed PMID: 17110507.

Pathophysiology of POTS

Cardiac Origins of POTS PDF

  • Fu Q, Vangundy TB, Galbreath MM, Shibata S, Jain M, Hastings JL, Bhella PS, Levine BD. Cardiac origins of the postural orthostatic tachycardia syndrome. J Am Coll Cardiol. 2010 Jun 22;55(25):2858-68. doi: 10.1016/j.jacc.2010.02.043. PubMed PMID: 20579544; PubMed Central PMCID: PMC2914315.

Sleep & Quality of Life

Sleep Quality and Diminished Quality of Life in POTS PDF

  • Bagai K, Song Y, Ling JF, Malow B, Black BK, Biaggioni I, Robertson D, Raj SR. Sleep disturbances and diminished quality of life in postural tachycardia syndrome. J Clin Sleep Med. 2011 Apr 15;7(2):204-10. PubMed PMID: 21509337; PubMed Central PMCID: PMC3077350.

Estimation of Sleep in POTS PDF

  • Bagai K, Wakwe CI, Malow B, Black BK, Biaggioni I, Paranjape SY, Orozco C, Raj SR. Estimation of sleep disturbances using wrist actigraphy in patients with postural tachycardia syndrome. Auton Neurosci. 2013 Oct;177(2):260-5. doi: 10.1016/j.autneu.2013.02.021. Epub 2013 Mar 26. PubMed PMID: 23538032; PubMed Central PMCID: PMC3700681.

Quality of Life in POTS

  • Benrud-Larson LM, Dewar MS, Sandroni P, Rummans TA, Haythornthwaite JA, Low PA. Quality of life in patients with postural tachycardia syndrome. Mayo Clin Proc. 2002 Jun;77(6):531-7. PubMed PMID: 12059122

Medications For POTS

Desmopressin in POTS PDF

  • Coffin ST, Black BK, Biaggioni I, Paranjape SY, Orozco C, Black PW, Dupont WD, Robertson D, Raj SR. Desmopressin acutely decreases tachycardia and improves symptoms in the postural tachycardia syndrome. Heart Rhythm. 2012 Sep;9(9):1484-90. doi: 10.1016/j.hrthm.2012.05.002. Epub 2012 May 3. PubMed PMID: 22561596; PubMed Central PMCID: PMC3419341.

Melatonin in POTS PDF

  • Green EA, Black BK, Biaggioni I, Paranjape SY, Bagai K, Shibao C, Okoye MC, Dupont WD, Robertson D, Raj SR. Melatonin reduces tachycardia in postural tachycardia syndrome: a randomized, crossover trial. Cardiovasc Ther. 2014 Jun;32(3):105-12. doi: 10.1111/1755-5922.12067. PubMed PMID: 24495468; PubMed Central PMCID: PMC3999238.

Effects of SSRI in POTS PDF

  • Mar PL, Raj V, Black BK, Biaggioni I, Shibao CA, Paranjape SY, Dupont WD, Robertson D, Raj SR. Acute hemodynamic effects of a selective serotonin reuptake inhibitor in postural tachycardia syndrome: a randomized, crossover trial. J Psychopharmacol. 2014 Feb;28(2):155-61. doi: 10.1177/0269881113512911. Epub 2013 Nov 13. PubMed PMID: 24227635; PubMed Central PMCID: PMC3956655.

Volume Loading and Pressor Agents in POTS

  • Jacob G, Shannon JR, Black B, Biaggioni I, Mosqueda-Garcia R, Robertson RM, Robertson D. Effects of volume loading and pressor agents in idiopathic orthostatic tachycardia. Circulation. 1997 Jul 15;96(2):575-80. PubMed PMID: 9244228.

Pyridostigmine in POTS PDF

  • Kanjwal K, Karabin B, Sheikh M, Elmer L, Kanjwal Y, Saeed B, Grubb BP. Pyridostigmine in the treatment of postural orthostatic tachycardia: a single-center experience. Pacing Clin Electrophysiol. 2011 Jun;34(6):750-5. doi: 10.1111/j.1540-8159.2011.03047.x. Epub 2011 Mar 16. PubMed PMID: 21410722.

Exercise in POTS

Exercise vs. Propanolol in POTS PDF

  • Fu Q, Vangundy TB, Shibata S, Auchus RJ, Williams GH, Levine BD. Exercise training versus propranolol in the treatment of the postural orthostatic tachycardia syndrome. Hypertension. 2011 Aug;58(2):167-75. doi: 10.1161/HYPERTENSIONAHA.111.172262. Epub 2011 Jun 20. PubMed PMID: 21690484; PubMed Central PMCID: PMC3142863.

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MCAD Journal Articles

MCAD Diagnosis and Treatment

MCAD Diagnostic Criteria PDF

  • Akin, Cem, Peter Valent, and Dean D. Metcalfe. “Mast Cell Activation Syndrome: Proposed Diagnostic Criteria: Towards a Global Classification for Mast Cell Disorders.” The Journal of allergy and clinical immunology 126.6 (2010): 1099–104.e4. PMC. Web. 20 Apr. 2018.

MCAD Workup and Therapeutic Options PDF

  • Molderings, Gerhard J et al. “Mast Cell Activation Disease: A Concise Practical Guide for Diagnostic Workup and Therapeutic Options.” Journal of Hematology & Oncology 4 (2011): 10. PMC. Web. 20 Apr. 2018.

In Depth Article on Medication for MCAD PDF

  • Molderings, G.J., Haenisch, B., Brettner, S. et al. Naunyn-Schmiedeberg's Arch Pharmacol (2016) 389: 671. https://doi.org/10.1007/s00210-016-1247-1

Allergic Reactions and the Nervous System PDF

Mast Cell Activation Disorder in Hyperadrenergic POTS Patients PDF

  • Shibao C, Arzubiaga C, Roberts LJ 2nd, Raj S, Black B, Harris P, Biaggioni I. Hyperadrenergic postural tachycardia syndrome in mast cell activation disorders. Hypertension. 2005 Mar;45(3):385-90. Epub 2005 Feb 14. PubMed PMID: 15710782.

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Orthostatic Hypotension and Neurocardiogenic Syncope Journal Articles

Orthostatic Hypotension

Preventing and Treating OH PDF

  • Figueroa JJ, Basford JR, Low PA. Preventing and treating orthostatic hypotension: As easy as A, B, C. Cleveland Clinic journal of medicine. 2010;77(5):298-306. doi:10.3949/ccjm.77a.09118.

Managed Care Approach to the Treatment of OH PDF

  • Isaacson SH. Managed care approach to the treatment of neurogenic orthostatic hypotension. Am J Manag Care. 2015 Oct;21(13 Suppl):s258-68. PubMed PMID: 26790110.

Preventing and treating orthostatic hypotension: As easy as A, B, C

  • FIGUEROA JJ, BASFORD JR, LOW PA. Preventing and treating orthostatic hypotension: As easy as A, B, C. Cleveland Clinic journal of medicine. 2010;77(5):298-306. doi:10.3949/ccjm.77a.09118.

NCS

Neurocardiogenic Syncope and Related Disorders of Orthostatic Intolerance PDF

  • Grubb BP. Neurocardiogenic syncope and related disorders of orthostatic intolerance. Circulation. 2005 Jun 7;111(22):2997-3006. Review. PubMed PMID: 15939833.

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MSA and PAF Journal Articles

MSA & PAF

Supine Hypertension and OH in Autonomic Failure PDF

  • Goldstein DS, Pechnik S, Holmes C, Eldadah B, Sharabi Y. Association between supine hypertension and orthostatic hypotension in autonomic failure. Hypertension. 2003 Aug;42(2):136-42. Epub 2003 Jun 30. Erratum in: Hypertension. 2003 Oct;43(4):e12. PubMed PMID: 12835329.

Multiple System Atrophy: A Clinical and Neuropathological Perspective PDF

  • Ubhi K, Low P, Masliah E. Multiple System Atrophy: A Clinical and Neuropathological Perspective. Trends in neurosciences. 2011;34(11):581-590. doi:10.1016/j.tins.2011.08.003.

Autonomic Dysfunction in Parkinsonian MSA vs. Parkinson’s Disease PDF

  • Roy S, Jaryal AK, Srivastava AK, Deepak KK. Cardiovagal Baroreflex Sensitivity in Parkinson’s Disease and Multiple-System Atrophy. Journal of Clinical Neurology (Seoul, Korea). 2016;12(2):218-223. doi:10.3988/jcn.2016.12.2.218.

Orthostatic Hypotension in MSA PDF

  • Sun Z, Jia D, Shi Y, et al. Prediction of orthostatic hypotension in multiple system atrophy and Parkinson disease. Scientific Reports. 2016;6:21649. doi:10.1038/srep21649.

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Ehlers-Danlos Syndromes Journal Articles

The American Journal of Medical Genetics has released a supplement issue containing 18 new articles on the Ehlers Danlos syndromes and related disorders. These papers were compiled by an International Consortium of over 90 researchers and clinicians from around the world who specialize in the Ehlers Danlos syndromes.

Topics include:

  • Expanded classifications to include 13 distinct types of Ehlers Danlos syndromes as well as the newly defined hypermobility spectrum disorders.

  • New diagnostic criteria for all types with management and care guidelines.

  • Information on co-morbid conditions and symptoms, including management and care guidelines.

  • Guidelines for specialists in Orthopedics, Physical Therapy, Cardiology, Gastroenterology, Neurology, Immunology, Psychiatry and Psychology, Dentistry, and Pain Management.

Ehlers-Danlos Syndromes

Nursing Management of Patients with Ehlers–Danlos Syndrome PDF

  • Anderson, L. Nursing management of patients with Ehlers-Danlos syndrome. American Journal of Nursing, 2015, 115(7), 38-46.

Anesthesia and Perioperative Care of EDS Patients PDF

  • Wiesmann, T., Castrori, M., Malfait, F., Wulf, H. Recommendations for anesthesia management in patients with Ehlers Danlos syndrome(s). Orphanet Journal of Rare Diseases (2014), 9(109), 1-9.

Coexistence of Ehlers-Danlos syndrome and multiple sclerosis PDF

  • Ozlece, H., Ilik, F., Huseyinoglu, N. Coexistence of Ehlers-Danlos syndrome and multiple sclerosis. Iran Journal of Neurology, 2015, 14(2), 116-117.

Ehlers-Danlos Syndrome and POTS PDF

  • Grigoriou, E., Bros, J., Dormans, J. Postural orthostatic tachycardia syndrome: association with Ehlers-Danlos syndrome and orthopedic considerations. Clinical Orthopedics and Related Research, 2015, 473(2), 722-728.

Neurological Manifestations of Ehlers-Danlos Syndrome PDF

  • Castori, M., Voermans, N. Neurological manifestations of Ehlers-Danlos Syndrome(s): a review. Iran Journal of Neurology, 2014, 13(4), 190-280.

Chronic Pain in EDS PDF

  • Scheper, M., de Vries, J., Verbunt, J., Engelbert, R. Chronic pain in hypermobility syndrome and Ehlers-Danlos syndrome (hypermobility type): it is a challenge. Journal of Pain Research (2015), 8, 591-601.

Pregnancy in EDS

Pregnancy Related Complications in VEDS PDF

  • Murray, M., Pepin, M., Peterson, S., Byers, P. Pregnancy-related deaths and complications in women with vascular Ehlers-Danlos syndrome. Genetics in Medicine (2014), 16, 874-880.

OB/GYN Complications in EDS PDF

  • Hurst, B., Lange, S., Kullstam, S., et al. Obstetric and gynecologic challenges in women with Ehlers-Danlos syndrome. Obstetric Gynecology, 2014, 123(3), 506-513.

hEDS During Pregnancy, Birth, & Beyond  PDF

  • Pezaro, S., Pearce, G., Reinhold, E. Hypermobile Ehlers-Danlos Syndrome during pregnancy, birth an beyond. British Journal of Midwifery, 2018, 26(4), 217-223.

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Gastroparesis Journal Articles

Overview of Gastroparesis

Overview of Gastroparesis Causes, Symptoms, Diagnosis, and Management PDF

  • Parkman, H., Camilleri, M., Farrugia, G. et al. Gastroparesis and functional dyspepsia: Excerpts from the AGA/ ANMS meeting. Neurogastroenterology Motility. 2010, 22(2), 113-133.

Gastroparesis Updates on Pathogenesis and Management PDF

  • Nanlong Liu and Thomas Abell. Gastroparesis Updates on Pathogensis and Management. Gut and Liver, Vol. 11, No. 5, September 2017, pp. 579-589

Nutrition Deficiencies with Gastroparesis​ - PDF

  • The NIDDK Gastroparesis Clinical Research Consortium (GpCRC). Dietary Intake and Nutritional Deficiencies in Patients with Diabetic or Idiopathic Gastroparesis. Gastroenterology. 2011 August ; 141(2): 486–498.e7. doi:10.1053/j.gastro.2011.04.045

Gastroparesis Diagnosis and Testing

Recommendations for Measuring Gastric Emptying PDF

  • Abell, T., Camilleri, M., Donohue, K., et al. Consensus recommendations for gastric emptying scitingraphy A joint report of the American Neurogastroenterology and Motility Society and the Society of Nuclear Medicine. Journal of Nuclear Medicine Technology, 2008, 36(1), 44-52.

Gastroparesis Treatment and Management

Diet, Drugs and Interventions for Gastroparesis - PDF

  • Camilleri, M. Novel Diet, Drugs, and Gastric Interventions for Gastroparesis. Clin Gastroenterol Hepatol. 2016 August ; 14(8): 1072–1080

Pyridostigmine for Autoimmune Gastrointestinal Dysmotility PDF

  • Pasha, S., Lunsford, T., Lennon,V. Autoimmune gastrointestinal dysmotility treated successfully with pyridostigmine. Gastroenterology, 2006,1(31), 1592-1596.

Clinical Guideline: Management of Gastroparesis PDF

  • Camilleri, M., Parkman, H., Shafi, M., et al. Clinical guideline: Management of gastroparesis. American Journal of Gastroenterology, 2013, 108(1), 18-38.

 

Botox Injections for Gastroparesis PDF

  • Ukleja, A., Tandon, K., Shah, K, et al. Endoscopic botox injections in therapy of refractory gastroparesis. World Journal of Gastrointestinal Endoscopy, 2015, 10(7), 790-798.

A Review of Gastric Electrical Stimulators (“gastric pacemaker”) for Gastroparesis PDF

  • Lal, N., Livemore, S., Dunne, D., et al. Gastric electrical stimulation with the enterra system: A systematic review. Gastroenterology research and practice, 2015, 1-9.

 

Ongoing Clinical Trials for Relamorelin

  • Chedid V and Camilleri M. Relamorelin for the treatment of gastrointestinal motility disorders. Expert Opin Investig Drugs. 2017 Oct;26(10):1189-1197. doi: 10.1080/13543784.2017.1373088. Epub 2017 Aug 31

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Neuropathy Journal Articles

Overview

Cleveland Clinic Overview of Small Fiber Neuropathy  PDF

  • Tavee, J, Zhou, L. Small fiber neuropathy: A burning problem. Cleveland Clinic Journal of Medicine, 2009, 76(5), 297-305.

Small Nerve Fiber Neuropathy Classifications, Diagnosis, and Treatment

  • Levine, T. Small Fiber Neuropathy: Disease Classification Beyond Pain and Burning. Journal of Central Nervous System Disease , 2018, Vol 10, 1-6

 

Conditions Associated with Small Nerve Fiber Neuropathy

  • de Greef, B, Hoeijmakers, J, Gorissen‐Brouwers, C, Geerts, M, Faber, C, Merkies, I. Associated Conditions in Small Nerve Fiber Neuropathy - a large cohort study and review of literature. Eur J Neurol. 2018 Feb; 25(2): 348–355.

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CRPS Journal Articles

Overview

Complex Regional Pain Syndrome PDF

  • Birklein F, Dimova V. Complex regional pain syndrome-up-to-date. Pain Rep. 2017 Oct 5;2(6):e624. doi: 10.1097/PR9.0000000000000624

New Hypothosis for Pathophysiology of CRPS

  • Russo M, Georgius P, Santarelli D. A new hypothesis for the pathophysiology of complex regional pain syndrome. Med Hypotheses. 2018 Oct;119:41-53. doi: 10.1016/j.mehy.2018.07.026

Body Perception and Sense Perception with CRPS

  • Brun C, Giorgi N, Pinard AM, Gagné M, McCabe CS, Mercier C. J. Exploring the Relationships Between Altered Body Perception, Limb Position Sense, and Limb Movement Sense in Complex Regional Pain Syndrome. Pain. 2018 Aug 9. pii: S1526-5900(18)30380-8. doi: 10.1016/j.jpain.2018.07.008

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