Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Autonomic Neuropathy Medication

  • Author: Steven D Arbogast, DO; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
 
Updated: May 05, 2016
 

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and prevent complications. Many of the medications used to treat dysautonomia are considered off label.

Next

Alpha-1 Agonists

Class Summary

These are used in orthostatic hypotension if simple measures yield no improvement.

Midodrine

 

Prodrug metabolized to desglymidodrine, a selective alpha1-adrenoreceptor agonist. Effects via arterioconstriction and venoconstriction.

Previous
Next

Mineralocorticoids

Class Summary

These agents can be used to treat orthostatic hypotension.

Fludrocortisone

 

Promotes increased reabsorption of sodium and loss of potassium at renal distal tubules.

Previous
Next

Anticholinergic agents

Class Summary

These agents are useful in cases of difficult bladder emptying.

Oxybutynin (Ditropan, Oxytrol, Gelnique)

 

Commonly used drug in bladder disorder. Known for anticholinergic-antispasmodic effects. Smooth muscle relaxing effect distal to cholinergic receptor site. Long-acting form available for qd dosing.

Tolterodine tartrate (Detrol)

 

Competitive muscarinic receptor antagonist for overactive bladder, but differs from other anticholinergic types because of selectivity for urinary bladder over salivary glands. High specificity for muscarinic receptors. Minimal activity or affinity for other neurotransmitter receptors and other potential targets (eg, calcium channels).

Previous
Next

Beta-adrenergic blocker

Class Summary

This agent can be used to treat orthostatic tachycardia in POTS patients.

Metoprolol (Lopressor, Toprol XL)

 

For treatment of orthostatic tachycardia. Inhibits beta-adrenergic input.

Previous
Next

Vasopressin analogs

Class Summary

Oral or nasal spray agents acting to prevent nocturnal urinary production.

Desmopressin acetate (DDAVP, Simate)

 

Vasopressin analogue without effect on V1 receptors responsible for vasopressin-induced vasoconstriction. Acts on V2 receptors at renal tubuli, increasing cellular permeability of collecting ducts, responsible for antidiuretic effect. Prevents nocturnal diuresis and elevated morning BP, resulting in renal water reabsorption. Nasal spray and tab (more convenient).

Previous
Next

Acetylcholinesterase inhibitor

Class Summary

This agent can be used to treat orthostatic hypotension or orthostatic tachycardia.

Pyridostigmine (Mestinon)

 

Increases acetylcholine neurotransmission at peripheral

autonomic ganglia, which likely increases peripheral vasoconstriction sympathetic nerve fiber transmission. May also increase vagal cardiac input in POTS patients.

Previous
Next

Phosphodiesterase inhibitors

Class Summary

These oral agents act peripherally to induce smooth muscle relaxation of the corpora cavernosa.

Sildenafil (Viagra)

 

Selective inhibitor of PDE5 that inactivates cGMP, allowing attenuation of the vasodilatory effect of NO. Effective in men with mild-to-moderate erectile dysfunction. Take on an empty stomach about 1 h before sexual activity. Sexual stimulation is necessary to activate response. The increased sensitivity for erections may last 24 h. Available as 25-, 50-, and 100-mg tabs.

Avanafil (Stendra)

 

Sexual stimulation causes nitric oxide to be released in the corpus cavernosum; nitric oxide activates the enzyme guanylate cyclase, which in turn increases cGMP levels; increase in cGMP levels causes smooth muscle relaxation.

Phosphodiesterase type 5 inhibitors enhance the effects of nitric oxide in smooth muscle relaxation of the corpus cavernosum by inhibiting the degradation of cGMP.

Tadalafil (Adcirca, Cialis)

 

Erectile dysfunction: Inhibits PDE-5, increasing cyclic guanosine monophosphate (cGMP) to allow smooth-muscle relaxation and inflow of blood into corpus cavernosum

Pulmonary arterial hypertension (PAH): Inhibits PDE-5, increasing cGMP to allow relaxation of pulmonary vascular smooth-muscle cells and vasodilation of pulmonary vasculature

Vardenafil (Levitra, Staxyn ODT)

 

Sexual stimulation causes nitric oxide to be released in corpus cavernosum, and nitric oxide activates guanylate cyclase, which in turn increases cyclic guanosine monophosphate (cGMP), thus causing smooth-muscle relaxation; PDE-5 inhibitors enhance smooth muscle-relaxing effects of nitric oxide in corpus cavernosum by inhibiting degradation of cGMP

Previous
Next

Neuromuscular blocker agent, toxin

Class Summary

Used in patients with hyperhydrosis localized to palmar or axillary region.

OnabotulinumtoxinA (Botox, Botox Cosmetic, Botulinum toxin)

 

One of several toxins produced by clostridium botulinum. Blocks neuromuscular transmission through a 3-step process: (1) Blockade of neuromuscular transmission; botulinum toxin type A (BTA) binds to the motor nerve terminal. The binding domain of the type A molecule appears to be the heavy chain, which is selective for cholinergic nerve terminals. (2) BTA is internalized via receptor-mediated endocytosis, a process in which the plasma membrane of the nerve cell invaginates around the toxin-receptor complex, forming a toxin-containing vesicle inside the nerve terminal. After internalization, the light chain of the toxin molecule, which has been demonstrated to contain the transmission-blocking domain, is released into the cytoplasm of the nerve terminal. (3) BTA blocks acetylcholine release by cleaving SNAP-25, a cytoplasmic protein that is located on the cell membrane and that is required for the release of this transmitter. The affected terminals are inhibited from stimulating muscle contraction. Toxin does not affect synthesis or storage of acetylcholine or conduction of electrical signals along the nerve fiber. Prevents calcium-dependent release of acetylcholine and produces a state of denervation at the neuromuscular junction and postganglionic sympathetic cholinergic nerves in the sweat glands.

Typically, a 24-72 h delay between administration of toxin and onset of clinical effects exists, which terminate in 2-6 mo.

This purified neurotoxin complex is a vacuum-dried form of purified BTA, which contains 5 ng of neurotoxin complex protein per 100 U.

BTA has to be reconstituted with 2 mL of 0.9% sodium chloride diluent. With this solution, each 0.1 mL results in 5 U dose. Patient should receive 5-10 injections per visit.

Must be reconstituted from vacuum-dried toxin into 0.9% sterile saline without preservative using manufacturer's instructions to provide injection volume of 0.1 mL; must be used within 4 h of storage in refrigerator at 2-8°C.

Preconstituted dry powder must be stored in freezer at < 5°C. Each injection produces an area of anhydrosis approximately 1.2 cm in diameter. Results in anhydrosis lasting 4-12 months.

Injections of botulinum toxin must be repeated at varying intervals to maintain long-term results.

Previous
Next

Colony-stimulating Factor

Class Summary

May be used in patients with orthostatic hypotension or POTS.

Epoetin alfa (Epogen, Procrit)

 

Purified glycoprotein produced from mammalian cells modified with gene coding for human erythropoietin (EPO). Amino acid sequence is identical to that of endogenous EPO. Biological activity mimics human urinary EPO, which stimulates division and differentiation of committed erythroid progenitor cells and induces release of reticulocytes from bone marrow into the blood stream.

Has been shown to increase the functional capacity of patients with MSA, particularly those who have the characteristic mild anemia associated with this disease. Up to 38% of patients with severe autonomic failure are anemic. Lack of sympathetic stimulation may lead to a decrease of erythropoietin production and development of anemia. Sympathetic impairment and low plasma norepinephrine levels have been found to correlate with severity of anemia. Therapy with recombinant erythropoietin, even low doses (25-50 units/kg body weight SC, 3 times a week) has successfully corrected anemia and improved upright BP.

Previous
Next

Anticholinergic agent

Class Summary

Used for hyperhidrosis.

Glycopyrrolate (Robinul, Glycate)

 

Acts in smooth muscle, CNS, and secretory glands to blocks action of acetylcholine at parasympathetic sites.

Previous
Next

Immune globulin

Class Summary

Used for autoimmune causes of autonomic neuropathy.

Immune globulin intravenous (Carimune NF, Gammagard S/D, Gamunex-C, Octagam)

 

Neutralize circulating myelin antibodies through antiidiotypic antibodies; down regulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; promotes remyelination; may increase CSF IgG (10%).

Previous
Next

Corticosteroids

Class Summary

Can be used if an inflammatory cause of the autonomic neuropathy is considered to be autoimmune in nature.

Prednisone (Rayos)

 

Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocyte and antibody production.

Previous
Next

Cholinergic agonist agents

Class Summary

These agents stimulate cholinergic receptors in the smooth muscle of the urinary bladder for stimulation of bladder emptying.

Bethanechol hydrochloride (Urecholine)

 

Used for selective stimulation of the bladder to produce contraction to initiate micturition and empty bladder. Most useful in bladder hypocontractility, if sphincters functional and coordinated. Rarely used because of GI stimulation and difficulty in timing effect.

Previous
 
 
Contributor Information and Disclosures
Author

Steven D Arbogast, DO Fellow, Neuromuscular Medicine, University Hospitals Case Medical Center, Cleveland

Steven D Arbogast, DO is a member of the following medical societies: American Academy of Neurology, American Osteopathic Association

Disclosure: Nothing to disclose.

Coauthor(s)

Bashar Katirji, MD, FACP Director, Neuromuscular Center and EMG Laboratory, The Neurological Institute, University Hospitals Case Medical Center; Professor of Neurology, Case Western Reserve University School of Medicine

Bashar Katirji, MD, FACP is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American College of Physicians, American Neurological Association

Disclosure: Nothing to disclose.

J Douglas Miles, MD, PhD Assistant Professor of Neuroscience, Marshall University School of Medicine, and Clinical Instructor of Neurology, Case Western Reserve University School of Medicine

J Douglas Miles, MD, PhD is a member of the following medical societies: American Academy of Neurology, American Medical Association, Society for Neuroscience

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Glenn Lopate, MD Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Consulting Staff, Department of Neurology, Barnes-Jewish Hospital

Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

Nicholas Lorenzo, MD, MHA, CPE Founding Editor-in-Chief, eMedicine Neurology; Founder and CEO/CMO, PHLT Consultants; Chief Medical Officer, MeMD Inc

Nicholas Lorenzo, MD, MHA, CPE is a member of the following medical societies: Alpha Omega Alpha, American Association for Physician Leadership, American Academy of Neurology

Disclosure: Nothing to disclose.

Additional Contributors

Paul E Barkhaus, MD Professor of Neurology and Physical Medicine and Rehabilitation, Department of Neurology, Medical College of Wisconsin; Section Chief, Neuromuscular and Autonomic Disorders, Department of Neurology, Director, ALS Program, Medical College of Wisconsin

Paul E Barkhaus, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

References
  1. Delahaye N, Rouzet F, Sarda L, et al. Impact of liver transplantation on cardiac autonomic denervation in familial amyloid polyneuropathy. Medicine (Baltimore). 2006. 85(4):229-238. [Medline].

  2. Davidson GL, Murphy SM, Polke JM, Laura M, Salih MA, Muntoni F, et al. Frequency of mutations in the genes associated with hereditary sensory and autonomic neuropathy in a UK cohort. J Neurol. 2012 Feb 1. [Medline].

  3. Bejaoui K, Wu C, Scheffler MD, et al. SPTLC1 is mutated in hereditary sensory neuropathy, type 1. Nat Genet. 2001 Mar. 27(3):261-2. [Medline].

  4. Low PA, Vernino S, Suarez G. Autonomic dysfunction in peripheral nerve disease. Muscle Nerve. 2003 Jun. 27(6):646-61. [Medline].

  5. Ohto T, Iwasaki N, Fujiwara J, et al. The evaluation of autonomic nervous function in a patient with hereditary sensory and autonomic neuropathy type IV with novel mutations of the TRKA gene. Neuropediatrics. 2004 Oct. 35(5):274-8. [Medline].

  6. Einarsdottir E, Carlsson A, Minde J, et al. A mutation in the nerve growth factor beta gene (NGFB) causes loss of pain perception. Hum Mol Genet. 2004 Apr 15. 13(8):799-805. [Medline].

  7. Low PA. Clinical autonomic disorders: evaluation and management. 2nd ed. New York: Lippincott Raven; 1997.

  8. Low PA, Dyck PJ, Lambert EH, et al. Acute panautonomic neuropathy. Ann Neurol. 1983 Apr. 13(4):412-7. [Medline].

  9. Vernino S, Low PA, Fealey RD, Stewart JD, Farrugia G, Lennon VA. Autoantibodies to ganglionic acetylcholine receptors in autoimmune autonomic neuropathies. N Engl J Med. 2000 Sep 21. 343(12):847-55. [Medline].

  10. Stewart JD, Low PA, Fealey RD. Distal small fiber neuropathy: results of tests of sweating and autonomic cardiovascular reflexes. Muscle Nerve. 1992 Jun. 15(6):661-5. [Medline].

  11. Emond D, Lebel M. Orthostatic hypotension and Holmes-Adie syndrome. Usefulness of the Valsalva ratio in the evaluation of baroreceptor dysfunction. J Hum Hypertens. 2002 Sep. 16(9):661-2. [Medline].

  12. Nolano M, Provitera V, Perretti A, Stancanelli A, Saltalamacchia AM, Donadio V, et al. Ross syndrome: a rare or a misknown disorder of thermoregulation? A skin innervation study on 12 subjects. Brain. 2006 Aug. 129:2119-31. [Medline].

  13. Low PA, Fealey RD, Sheps SG, Su WP, Trautmann JC, Kuntz NL. Chronic idiopathic anhidrosis. Ann Neurol. 1985 Sep. 18(3):344-8. [Medline].

  14. Benson MD, Kincaid JC. The molecular biology and clinical features of amyloid neuropathy. Muscle Nerve. 2007 Oct. 36(4):411-23. [Medline].

  15. Kyle RA, Greipp PR. Amyloidosis (AL). Clinical and laboratory features in 229 cases. Mayo Clin Proc. 1983 Oct. 58(10):665-83. [Medline].

  16. Low PA, Novak V, Spies JM, et al. Cerebrovascular regulation in the postural orthostatic tachycardia syndrome (POTS). Am J Med Sci. 1999 Feb. 317(2):124-33. [Medline].

  17. Novak V, Novak P, Opfer-Gehrking TL, et al. Clinical and laboratory indices that enhance the diagnosis of postural tachycardia syndrome. Mayo Clin Proc. 1998 Dec. 73(12):1141-50. [Medline].

  18. Zochodne DW. Diabetic neuropathies: features and mechanisms. Brain Pathol. 1999 Apr. 9(2):369-91. [Medline].

  19. Vinik AI, Freeman R, Erbas T. Diabetic autonomic neuropathy. Semin Neurol. 2003 Dec. 23(4):365-72. [Medline].

  20. Clements RS Jr, Flint MA. Coping with autonomic neuropathy. J Diabet Complications. 1988 Jul-Sep. 2(3):130-2. [Medline].

  21. Supriya Simon A, Dinesh Roy D, Jayapal V, Vijayakumar T. Somatic DNA damages in cardiovascular autonomic neuropathy. Indian J Clin Biochem. 2011 Jan. 26(1):50-6. [Medline]. [Full Text].

  22. Semra YK, Wang M, Peat NJ, et al. Selective susceptibility of different populations of sympathetic neurons to diabetic neuropathy in vivo is reflected by increased vulnerability to oxidative stress in vitro. Neurosci Lett. 2006 Oct 30. 407(3):199-204. [Medline].

  23. Zochodne DW. The autonomic nervous system in peripheral neuropathies. Handbook of Clinical Neurology. 2000. 75(31):681-712.

  24. Maheshwari A, Thomas A, Thuluvath PJ. Patients with autonomic neuropathy are more likely to develop hepatic encephalopathy. Dig Dis Sci. 2004 Oct. 49(10):1584-8. [Medline].

  25. Beitzke M, Pfister P, Fortin J, Skrabal F. Autonomic dysfunction and hemodynamics in vitamin B12 deficiency. Auton Neurosci. 2002 Apr 18. 97(1):45-54. [Medline].

  26. Koike H, Sobue G. Alcoholic neuropathy. Curr Opin Neurol. 2006 Oct. 19(5):481-6. [Medline].

  27. Duray PH. Histopathology of clinical phases of human Lyme disease. Rheum Dis Clin North Am. 1989 Nov. 15(4):691-710. [Medline].

  28. Glück T, Degenhardt E, Schölmerich J, Lang B, Grossmann J, Straub RH. Autonomic neuropathy in patients with HIV: course, impact of disease stage, and medication. Clin Auton Res. 2000 Feb. 10(1):17-22. [Medline].

  29. Cohen JA, Laudenslager M. Autonomic nervous system involvement in patients with human immunodeficiency virus infection. Neurology. 1989 Aug. 39(8):1111-2. [Medline].

  30. Fernandez A, Hontebeyrie M, Said G. Autonomic neuropathy and immunological abnormalities in Chagas' disease. Clin Auton Res. 1992 Dec. 2(6):409-12. [Medline].

  31. Pentreath VW. Royal Society of Tropical Medicine and Hygiene Meeting at Manson House, London, 19 May 1994. Trypanosomiasis and the nervous system. Pathology and immunology. Trans R Soc Trop Med Hyg. 1995 Jan-Feb. 89(1):9-15. [Medline].

  32. Pickett JB 3rd. AAEE case report #16: Botulism. Muscle Nerve. 1988 Dec. 11(12):1201-5. [Medline].

  33. Idiaquez J. Autonomic dysfunction in diphtheritic neuropathy. J Neurol Neurosurg Psychiatry. 1992 Feb. 55(2):159-61. [Medline].

  34. Scollard DM. The biology of nerve injury in leprosy. Lepr Rev. 2008 Sep. 79(3):242-53. [Medline].

  35. Kyriakidis MK, Noutsis CG, Robinson-Kyriakidis CA, Venetsianos PJ, Vyssoulis GP, Toutouzas PC, et al. Autonomic neuropathy in leprosy. Int J Lepr Other Mycobact Dis. 1983 Sep. 51(3):331-5. [Medline].

  36. Gibbons CH, Freeman R. Autonomic neuropathy and coeliac disease. J Neurol Neurosurg Psychiatry. 2005 Apr. 76(4):579-81. [Medline].

  37. Tursi A, Giorgetti GM, Iani C. Peripheral Neurological Disturbances, Autonomic Dysfunction, and Antineuronal Antibodies in Adult Celiac Disease Before and After a Gluten-Free Diet. Dig Dis Sci. 2006 Sep 12; [Epub ahead of print]. [Medline].

  38. Gemignani F, Marbini A, Pavesi G, Di Vittorio S, Manganelli P, Cenacchi G, et al. Peripheral neuropathy associated with primary Sjögren's syndrome. J Neurol Neurosurg Psychiatry. 1994 Aug. 57(8):983-6. [Medline].

  39. Zochodne DW. Autonomic involvement in Guillain-Barré syndrome: a review. Muscle Nerve. 1994 Oct. 17(10):1145-55. [Medline].

  40. Panegyres PK, Mastaglia FL. Guillain-Barre syndrome with involvement of the central and autonomic nervous systems. Med J Aust. 1989 Jun 5. 150(11):655-9. [Medline].

  41. O'Suilleabhain P, Low PA, Lennon VA. Autonomic dysfunction in the Lambert-Eaton myasthenic syndrome: serologic and clinical correlates. Neurology. 1998 Jan. 50(1):88-93. [Medline].

  42. Sillevis Smitt P, Grefkens J, de Leeuw B, et al. Survival and outcome in 73 anti-Hu positive patients with paraneoplastic encephalomyelitis/sensory neuronopathy. J Neurol. 2002 Jun. 249(6):745-53. [Medline].

  43. Zenone T. [Autoimmunity and cancer: paraneoplastic neurological syndromes associated with small cell cancer]. Bull Cancer. 1992. 79(9):837-53. [Medline].

  44. Yu Z, Kryzer TJ, Griesmann GE, et al. CRMP-5 neuronal autoantibody: marker of lung cancer and thymoma-related autoimmunity. Ann Neurol. 2001 Feb. 49(2):146-54. [Medline].

  45. Straub RH, Antoniou E, Zeuner M, et al. Association of autonomic nervous hyperreflexia and systemic inflammation in patients with Crohn's disease and ulcerative colitis. J Neuroimmunol. 1997 Dec. 80(1-2):149-57. [Medline].

  46. Rose KM, Eigenbrodt ML, Biga RL, Couper DJ, Light KC, Sharrett AR, et al. Orthostatic hypotension predicts mortality in middle-aged adults: the Atherosclerosis Risk In Communities (ARIC) Study. Circulation. 2006 Aug 15. 114(7):630-6. [Medline].

  47. Suarez GA, Opfer-Gehrking TL, Offord KP, et al. The Autonomic Symptom Profile: a new instrument to assess autonomic symptoms. Neurology. 1999 Feb. 52(3):523-8. [Medline].

  48. Watkins PJ. Diabetic diarrhea, gastroparesis, and gustatory sweating. Dyck PJ, Thomas PK, Asbury AK, et al, eds. Diabetic Neuropathy. Philadelphia: WB Saunders Co; 1987. 199-200.

  49. Bharucha AE, Camilleri M, Low PA, Zinsmeister AR. Autonomic dysfunction in gastrointestinal motility disorders. Gut. 1993 Mar. 34(3):397-401. [Medline].

  50. Törnblom H. Treatment of gastrointestinal autonomic neuropathy. Diabetologia. 2016 Mar. 59 (3):409-13. [Medline].

  51. England JD, Gronseth GS, Franklin G, Carter GT, Kinsella LJ, Cohen JA, et al. Practice Parameter: evaluation of distal symmetric polyneuropathy: role of autonomic testing, nerve biopsy, and skin biopsy (an evidence-based review). Report of the American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Academy of Physical Medicine and Rehabilitation. Neurology. 2009 Jan 13. 72(2):177-84. [Medline].

  52. Foss CH, Vestbo E, Frøland A, Gjessing HJ, Mogensen CE, Damsgaard EM. Autonomic neuropathy in nondiabetic offspring of type 2 diabetic subjects is associated with urinary albumin excretion rate and 24-h ambulatory blood pressure: the Fredericia Study. Diabetes. 2001 Mar. 50(3):630-6. [Medline].

  53. Fealey RD. Thermoregulatory sweat test. Low PA, ed. Clinical Autonomic Disorders: Evaluation and Management. 2nd ed. Philadelphia: Lippincott-Raven; 1997. 245-57.

  54. Yarnitsky D, Sprecher E. Thermal testing: normative data and repeatability for various test algorithms. J Neurol Sci. 1994 Aug. 125(1):39-45. [Medline].

  55. Davies DR, Smith SE. Pupil abnormality in amyloidosis with autonomic neuropathy. J Neurol Neurosurg Psychiatry. 1999 Dec. 67(6):819-22. [Medline].

  56. Gibbons CH, Illigens BM, Centi J, Freeman R. QDIRT: quantitative direct and indirect test of sudomotor function. Neurology. 2008 Jun 10. 70(24):2299-304. [Medline].

  57. Madersbacher HG. Neurogenic bladder dysfunction. Curr Opin Urol. 1999 Jul. 9(4):303-7. [Medline].

  58. Ascaso JF, Herreros B, Sanchiz V, et al. Oesophageal motility disorders in type 1 diabetes mellitus and their relation to cardiovascular autonomic neuropathy. Neurogastroenterol Motil. 2006. 18(9):813-822. [Medline].

  59. Bissinger A, Grycewicz T, Grabowicz W, Lubinski A. The effect of diabetic autonomic neuropathy on P-wave duration, dispersion and atrial fibrillation. Arch Med Sci. 2011 Oct. 7(5):806-12. [Medline]. [Full Text].

  60. Pavy-Le Traon A, Fontaine S, Tap G, Guidolin B, Senard JM, Hanaire H. Cardiovascular autonomic neuropathy and other complications in type 1 diabetes. Clin Auton Res. 2010 Jun. 20(3):153-60. [Medline].

  61. Ohlsson B, Melander O, Thorsson O, et al. Oesophageal dysmotility, delayed gastric emptying and autonomic neuropathy correlate to disturbed glucose homeostasis. Diabetologia. 2006 Sep. 49(9):2010-4. [Medline].

  62. Devigili G, Tugnoli V, Penza P, Camozzi F, Lombardi R, Melli G, et al. The diagnostic criteria for small fibre neuropathy: from symptoms to neuropathology. Brain. 2008 Jul. 131:1912-25. [Medline].

  63. Singer W, Spies JM, McArthur J, et al. Prospective evaluation of somatic and autonomic small fibers in selected autonomic neuropathies. Neurology. 2004 Feb 24. 62(4):612-8. [Medline].

  64. Nolano M, Provitera V, Crisci C, et al. Quantification of myelinated endings and mechanoreceptors in human digital skin. Ann Neurol. 2003 Aug. 54(2):197-205. [Medline].

  65. Nolano M, Crisci C, Santoro L, et al. Absent innervation of skin and sweat glands in congenital insensitivity to pain with anhidrosis. Clin Neurophysiol. 2000 Sep. 111(9):1596-601. [Medline].

  66. Klein CM, Vernino S, Lennon VA, et al. The spectrum of autoimmune autonomic neuropathies. Ann Neurol. 2003 Jun. 53(6):752-8. [Medline].

  67. Low PA. Composite autonomic scoring scale for laboratory quantification of generalized autonomic failure. Mayo Clin Proc. 1993 Aug. 68(8):748-52. [Medline].

  68. Jordan J, Shannon JR, Black BK, et al. The pressor response to water drinking in humans : a sympathetic reflex?. Circulation. 2000 Feb 8. 101(5):504-9. [Medline].

  69. van Lieshout JJ, ten Harkel AD, Wieling W. Physical manoeuvres for combating orthostatic dizziness in autonomic failure. Lancet. 1992 Apr 11. 339(8798):897-8. [Medline].

  70. Thieben MJ, Sandroni P, Sletten DM, Benrud-Larson LM, Fealey RD, Vernino S, et al. Postural orthostatic tachycardia syndrome: the Mayo clinic experience. Mayo Clin Proc. 2007 Mar. 82(3):308-13. [Medline].

  71. Freeman R. Current pharmacologic treatment for orthostatic hypotension. Clin Auton Res. 2008 Mar. 18 Suppl 1:14-8. [Medline].

  72. Low PA. Autonomic neuropathies. Curr Opin Neurol. 1998 Oct. 11(5):531-7. [Medline].

  73. Singer W, Opfer-Gehrking TL, Nickander KK, Hines SM, Low PA. Acetylcholinesterase inhibition in patients with orthostatic intolerance. J Clin Neurophysiol. 2006 Oct. 23(5):476-81. [Medline].

  74. Grubb BP. Neurocardiogenic syncope and related disorders of orthostatic intolerance. Circulation. 2005 Jun 7. 111(22):2997-3006. [Medline].

  75. Gordon VM, Opfer-Gehrking TL, Novak V, Low PA. Hemodynamic and symptomatic effects of acute interventions on tilt in patients with postural tachycardia syndrome. Clin Auton Res. 2000 Feb. 10(1):29-33. [Medline].

  76. Winkler AS, Landau S, Watkins PJ. Erythropoietin treatment of postural hypotension in anemic type 1 diabetic patients with autonomic neuropathy: a case study of four patients. Diabetes Care. 2001 Jun. 24(6):1121-3. [Medline].

  77. Mathias CJ, Fosbraey P, da Costa DF, Thornley A, Bannister R. The effect of desmopressin on nocturnal polyuria, overnight weight loss, and morning postural hypotension in patients with autonomic failure. Br Med J (Clin Res Ed). 1986 Aug 9. 293(6543):353-4. [Medline].

  78. Dalakas MC. The use of intravenous immunoglobulin in the treatment of autoimmune neuromuscular diseases: evidence-based indications and safety profile. Pharmacol Ther. 2004 Jun. 102(3):177-93. [Medline].

  79. Schroeder C, Vernino S, Birkenfeld AL, Tank J, Heusser K, Lipp A, et al. Plasma exchange for primary autoimmune autonomic failure. N Engl J Med. 2005 Oct 13. 353(15):1585-90. [Medline].

  80. Modoni A, Mirabella M, Madia F, Sanna T, Lanza G, Tonali PA, et al. Chronic autoimmune autonomic neuropathy responsive to immunosuppressive therapy. Neurology. 2007 Jan 9. 68(2):161-2. [Medline].

  81. Shotton HR, Adams A, Lincoln J. Effect of aminoguanidine treatment on diabetes-induced changes in the myenteric plexus of rat ileum. Auton Neurosci. 2006 Sep 18 [Epub ahead of print]. [Medline].

  82. Klein CM. Evaluation and management of autonomic nervous system disorders. Semin Neurol. 2008 Apr. 28(2):195-204. [Medline].

  83. Grunfeld A, Murray CA, Solish N. Botulinum toxin for hyperhidrosis: a review. Am J Clin Dermatol. 2009. 10(2):87-102. [Medline].

  84. Monteiro E, Perdigoto R, Furtado AL. Liver transplantation for familial amyloid polyneuropathy. Hepatogastroenterology. 1998 Sep-Oct. 45(23):1375-80. [Medline].

  85. Tashima K, Ando Y, Terazaki H, et al. Outcome of liver transplantation for transthyretin amyloidosis: follow-up of Japanese familial amyloidotic polyneuropathy patients. J Neurol Sci. 1999 Dec 1. 171(1):19-23. [Medline].

  86. Antonelli Incalzi R, Fuso L, Pitocco D, et al. Decline of neuroadrenergic bronchial innervation and respiratory function in type 1 diabetes mellitus: a longitudinal study. Diabetes Metab Res Rev. 2006 Oct 2. [Medline].

  87. Dawkins JL, Hulme DJ, Brahmbhatt SB, et al. Mutations in SPTLC1, encoding serine palmitoyltransferase, long chain base subunit-1, cause hereditary sensory neuropathy type I. Nat Genet. 2001 Mar. 27(3):309-12. [Medline].

  88. El-Atat FA, McFarlane SI, Sowers JR, Bigger JT. Sudden cardiac death in patients with diabetes. Curr Diab Rep. 2004 Jun. 4(3):187-93. [Medline].

  89. Holland NR, Crawford TO, Hauer P, et al. Small-fiber sensory neuropathies: clinical course and neuropathology of idiopathic cases. Ann Neurol. 1998 Jul. 44(1):47-59. [Medline].

  90. Kamalakannan D, Baskar V, Singh BM. Severe and disabling diabetic autonomic neuropathy: a case report. J Diabetes Complications. 2004 Mar-Apr. 18(2):126-8. [Medline].

  91. Kudat H, Akkaya V, Sozen AB, et al. Heart rate variability in diabetes patients. J Int Med Res. 2006 May-Jun. 34(3):291-6. [Medline].

  92. Low PA, Caskey PE, Tuck RR, et al. Quantitative sudomotor axon reflex test in normal and neuropathic subjects. Ann Neurol. 1983 Nov. 14(5):573-80. [Medline].

  93. Lyu RK, Tang LM, Wu YR, Chen ST. Cardiovascular autonomic function and sympathetic skin response in chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 2002 Nov. 26(5):669-72. [Medline].

  94. Toth C, Zochodne DW. Other autonomic neuropathies. Semin Neurol. 2003 Dec. 23(4):373-80. [Medline].

  95. Zochodne DW, Auer R, Fritzler MJ. Longstanding ataxic demyelinating polyneuronopathy with a novel autoantibody. Neurology. 2003 Jan 14. 60(1):127-9. [Medline].

 
Previous
Next
 
Table. Types of HSAN
HSAN Mode of Inheritance Onset Symptoms Signs
Type I Autosomal dominant, point mutations in SPT, 9q22.1-9q22.3 Second decade of life Distal lower-limb involvement, ulceration of the feet, particularly the soles Low sensory action potential amplitude
Type II, Morvan disease Autosomal recessive Congenital onset Pansensory loss of upper and lower limbs, also trunk and forehead; early ulcers Loss of myelinated and unmyelinated fibers
Type III, Riley-Day syndrome or familial dysautonomia) Autosomal recessive, 9q31 Childhood onset, predominantly Ashkenazi Jews Pallor in infancy, irregularities in temperature and blood pressure; Difficulties in eating and swallowing Absence of unmyelinated fibers
Type IV Autosomal recessive, 1q21-1q22 Congenital onset Widespread anhidrosis, lost sense of pain, mental retardation Loss of myelinated and small unmyelinated fibers
Type V Autosomal recessive Congenital onset Pain insensitivity in extremities Not applicable
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.