Paraneoplastic Autonomic Neuropathy Follow-up

  • Author: Daniel Mordechai Goldenholz, MD, PhD; Chief Editor: Tarakad S Ramachandran, MBBS, FRCP(C), FACP   more...
 
Updated: Dec 10, 2010
 

Further Inpatient Care

Typically, the disorder is progressive, although stabilization with antineoplastic therapy has been reported. Subsequent follow-up is devoted to assessing adequacy of blood pressure support and bowel and bladder management.

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Further Outpatient Care

Custodial nursing home care may be needed if the autonomic failure is severe, and patients are bedridden and completely disabled.

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Inpatient & Outpatient Medications

Medications are the same as those used in inpatient care, except doses are adjusted with time and disease progression. See article Idiopathic Orthostatic Hypotension and Other Autonomic Failure Syndromes for specific suggestions.

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Deterrence/Prevention

No known method is effective in deterring or preventing occurrence of autonomic paraneoplastic failure, except to prevent exposure to known carcinogens such as tobacco smoke.

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Complications

Sudden death, often due to cardiac causes, can occur. Intestinal pseudo-obstruction and urinary tract infections from incomplete bladder emptying are possible. Overheating due to reduced sweat function can occur in hot temperatures or with physical exertion.

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Prognosis

Prognosis is generally poor. Prompt and effective treatment of the underlying malignancy may arrest progression of autonomic dysfunction, but in many cases, no improvement occurs. Survival is dependent on underlying cancer, patient age, and extent of nervous system involvement. The median survival of all patients with paraneoplastic syndromes has been estimated to be 1 to 3 years.[7] The 2 major exceptions are LEMS and NMDA receptor antibody disease. In Lambert-Eaton syndrome, effective treatment often results in remission of the mild autonomic symptoms and better than expected survival.[37, 38, 39] In NMDA receptor antibody disease, treatment results in recovery 75% of the time; however, this disease is still poorly characterized and likely underdiagnosed, so these numbers may change as more data become available.[20]

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Contributor Information and Disclosures
Author

Daniel Mordechai Goldenholz, MD, PhD  Resident Physician, Department of Neurology, University of California Davis Medical Center

Daniel Mordechai Goldenholz, MD, PhD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Coauthor(s)

Bjorn E Oskarsson, MD  Assistant Professor, Department of Neurology, University of California Davis

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

Disclosure: Nothing to disclose.

Dianna Quan, MD  Associate Professor of Neurology, Director, Electromyography Laboratory, University of Colorado School of Medicine

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

Disclosure: e-medicine Honoraria Other

Ronald G Wiley, MD, PhD  Chief, Professor, Department of Neurology, Veterans Affairs Medical Center, Vanderbilt University

Ronald G Wiley, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Association for the Advancement of Science, New York Academy of Sciences, and Society for Neuroscience

Disclosure: Nothing to disclose.

Specialty Editor Board

Amy A Pruitt, MD  Associate Professor of Neurology, University of Pennsylvania School of Medicine; Attending Neurologist, Hospital of the University of Pennsylvania

Amy A Pruitt, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

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

Disclosure: Medscape Salary Employment

Jorge Kattah, MD  Head, Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria

Jorge Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and New York Academy of Sciences

Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Tarakad S Ramachandran, MBBS, FRCP(C), FACP  Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, FRCP(C), FACP is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners, American College of International Physicians, American College of Managed Care Medicine, American College of Physicians, American Heart Association, American Stroke Association, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, and Royal Society of Medicine

Disclosure: Abbott Labs None None; Teva Marion None None; Boeringer-Ingelheim Honoraria Speaking and teaching

References

  1. Graus F, Delattre JY, Antoine JC, et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry. Aug 2004;75(8):1135-40. [Medline].

  2. Anderson NE, Rosenblum MK, Graus F. Autoantibodies in paraneoplastic syndromes associated with small-cell lung cancer. Neurology. Sep 1988;38(9):1391-8. [Medline].

  3. Elrington GM, Murray NM, Spiro SG. Neurological paraneoplastic syndromes in patients with small cell lung cancer. A prospective survey of 150 patients. J Neurol Neurosurg Psychiatry. Sep 1991;54(9):764-7. [Medline].

  4. Veilleux M, Bernier JP, Lamarche JB. Paraneoplastic encephalomyelitis and subacute dysautonomia due to an occult atypical carcinoid tumour of the lung. Can J Neurol Sci. Aug 1990;17(3):324-8. [Medline].

  5. Blaes F, Strittmatter M, Schwamborn J, et al. Antineuronal antibody-associated paraneoplastic neuropathy in Hodgkin's disease. Eur J Neurol. Jan 1998;5(1):109-112. [Medline].

  6. Ficker DM, Hammack JE. Primary central nervous system lymphoma presenting as autonomic dysfunction. J Neurooncol. 1995;23(3):245-8. [Medline].

  7. Candler PM, Hart PE, Barnett M, et al. A follow up study of patients with paraneoplastic neurological disease in the United Kingdom. J Neurol Neurosurg Psychiatry. Oct 2004;75(10):1411-5. [Medline].

  8. Altermatt HJ, Rodriguez M, Scheithauer BW, Lennon VA. Paraneoplastic anti-Purkinje and type I anti-neuronal nuclear autoantibodies bind selectively to central, peripheral, and autonomic nervous system cells. Lab Invest. Oct 1991;65(4):412-20. [Medline].

  9. Pittock SJ, Kryzer TJ, Lennon VA. Paraneoplastic antibodies coexist and predict cancer, not neurological syndrome. Ann Neurol. Nov 2004;56(5):715-9. [Medline].

  10. Panegyres PK, Reading MC, Esiri MM. The inflammatory reaction of paraneoplastic ganglionitis and encephalitis: an immunohistochemical study. J Neurol. Feb 1993;240(2):93-7. [Medline].

  11. Vernino S, Adamski J, Kryzer TJ. Neuronal nicotinic ACh receptor antibody in subacute autonomic neuropathy and cancer-related syndromes. Neurology. Jun 1998;50(6):1806-13. [Medline].

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

  13. Freeman R. Autonomic peripheral neuropathy. Lancet. Apr 2-8 2005;365(9466):1259-70. [Medline].

  14. Peltier AC, Black BK, Raj SR, Donofrio P, Robertson D, Biaggioni I. Coexistent autoimmune autonomic ganglionopathy and myasthenia gravis associated with non-small-cell lung cancer. Muscle Nerve. Mar 2010;41(3):416-9. [Medline].

  15. Lorusso L, Hart IK, Ferrari D, Ngonga GK, Gasparetto C, Ricevuti G. Autonomic paraneoplastic neurological syndromes. Autoimmun Rev. Jan 2007;6(3):162-8. [Medline].

  16. de Beukelaar JW, Sillevis Smitt PA. Managing paraneoplastic neurological disorders. Oncologist. Mar 2006;11(3):292-305. [Medline].

  17. Lucchinetti CF, Kimmel DW, Lennon VA. Paraneoplastic and oncologic profiles of patients seropositive for type 1 antineuronal nuclear autoantibodies. Neurology. Mar 1998;50(3):652-7. [Medline].

  18. Camdessanche JP, Antoine JC, Honnorat J. Paraneoplastic peripheral neuropathy associated with anti-Hu antibodies. A clinical and electrophysiological study of 20 patients. Brain. Jan 2002;125(Pt 1):166-75. [Medline].

  19. Vernino S. Antibody testing as a diagnostic tool in autonomic disorders. Clin Auton Res. Feb 2009;19(1):13-9. [Medline].

  20. Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. Dec 2008;7(12):1091-8. [Medline].

  21. Mahadeva B, Phillips LH 2nd, Juel VC. Autoimmune disorders of neuromuscular transmission. Semin Neurol. Apr 2008;28(2):212-27. [Medline].

  22. Vernino S, Lennon VA. New Purkinje cell antibody (PCA-2): marker of lung cancer-related neurological autoimmunity. Ann Neurol. Mar 2000;47(3):297-305. [Medline].

  23. Tschernatsch M, Klotz M, Probst C, Hosch J, Valtorta F, Diener M, et al. Synaptophysin is an autoantigen in paraneoplastic neuropathy. J Neuroimmunol. Jun 15 2008;197(1):81-6. [Medline].

  24. Tan KM, Lennon VA, Klein CJ, Boeve BF, Pittock SJ. Clinical spectrum of voltage-gated potassium channel autoimmunity. Neurology. May 13 2008;70(20):1883-90. [Medline].

  25. Tan KM, Lennon VA, Klein CJ, Boeve BF, Pittock SJ. Clinical spectrum of voltage-gated potassium channel autoimmunity. Neurology. May 13 2008;70(20):1883-90. [Medline].

  26. Dhamija R, Tan KM, Pittock SJ, Foxx-Orenstein A, Benarroch E, Lennon VA. Serologic profiles aiding the diagnosis of autoimmune gastrointestinal dysmotility. Clin Gastroenterol Hepatol. Sep 2008;6(9):988-92. [Medline].

  27. Duddy ME, Baker MR. Stiff person syndrome. Front Neurol Neurosci. 2009;26:147-65. [Medline].

  28. Graus F, Saiz A, Dalmau J. Antibodies and neuronal autoimmune disorders of the CNS. J Neurol. Apr 2010;257(4):509-17. [Medline].

  29. Tschernatsch M, Klotz M, Probst C, Hosch J, Valtorta F, Diener M. Synaptophysin is an autoantigen in paraneoplastic neuropathy. J Neuroimmunol. Jun 15 2008;197(1):81-6. [Medline].

  30. Byrne T, Mason WP, Posner JB, et al. Spontaneous neurological improvement in anti-Hu associated encephalomyelitis. J Neurol Neurosurg Psychiatry. Mar 1997;62(3):276-8. [Medline].

  31. Chalk CH, Windebank AJ, Kimmel DW, McManis PG. The distinctive clinical features of paraneoplastic sensory neuronopathy. Can J Neurol Sci. Aug 1992;19(3):346-51. [Medline].

  32. Chinn JS, Schuffler MD. Paraneoplastic visceral neuropathy as a cause of severe gastrointestinal motor dysfunction. Gastroenterology. Nov 1988;95(5):1279-86. [Medline].

  33. Dalmau J, Graus F, Rosenblum MK. Anti-Hu--associated paraneoplastic encephalomyelitis/sensory neuronopathy. A clinical study of 71 patients. Medicine (Baltimore). Mar 1992;71(2):59-72. [Medline].

  34. Titulaer MJ, Soffietti R, Dalmau J, Gilhus NE, Giometto B, Graus F, et al. Screening for tumours in paraneoplastic syndromes: report of an EFNS Task Force. Eur J Neurol. Sep 29 2010;[Medline].

  35. Oh SJ, Dropcho EJ, Claussen GC. Anti-Hu-associated paraneoplastic sensory neuropathy responding to early aggressive immunotherapy: report of two cases and review of literature. Muscle Nerve. Dec 1997;20(12):1576-82. [Medline].

  36. Dhamija R, Tan KM, Pittock SJ, Foxx-Orenstein A, Benarroch E, Lennon VA. Serologic profiles aiding the diagnosis of autoimmune gastrointestinal dysmotility. Clin Gastroenterol Hepatol. Sep 2008;6(9):988-92. [Medline].

  37. Khurana RK, Koski CL, Mayer RF. Autonomic dysfunction in Lambert-Eaton myasthenic syndrome. J Neurol Sci. May 1988;85(1):77-86. [Medline].

  38. Maddison P, Lang B. Paraneoplastic neurological autoimmunity and survival in small-cell lung cancer. J Neuroimmunol. Sep 15 2008;201-202:159-62. [Medline].

  39. Mamdani MB, Walsh RL, Rubino FA. Autonomic dysfunction and Eaton Lambert syndrome. J Auton Nerv Syst. Apr 1985;12(4):315-20. [Medline].

  40. Block MS, Vassallo R. Lack of FDG uptake in small cell carcinoma associated with ANNA-1 positive paraneoplastic autonomic neuropathy. J Thorac Oncol. May 2008;3(5):542-4. [Medline].

 
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Paraneoplastic autonomic neuropathy. Histopathology of peripheral nerve and sympathetic ganglion from a patient with autonomic failure, oat-cell carcinoma of the lung, and positive anti-HU antibody titer. (a) Peripheral nerve in longitudinal section stained with Luxol fast blue-periodic acid-Schiff (PAS) showing scattered wallerian degeneration (arrowheads). (b) Low-power view of a paravertebral sympathetic ganglion stained with hematoxylin and eosin (H&E). Arrowhead indicates perivascular mononuclear infiltrates. (c) High-power view of the same sympathetic ganglion showing degenerating neurons (single arrowheads) and mononuclear infiltrates (double arrowhead). Magnification bars in a and b indicate 100 mm; c is 50 mm.
Paraneoplastic autonomic neuropathy. Hematoxylin and eosin (H&E)–stained sections from dorsal root ganglion showing the hallmark histopathology of anti-HU disease; a and c are from a healthy patient; b and d are from a patient with autonomic failure, oat-cell carcinoma of the lung, and positive titer of anti-HU antibodies. The arrowheads in b and d indicate degenerating sensory neurons. Also note the interstitial hypercellularity and decreased numbers of neurons in b and d. Magnification bar in b indicates 100 mm and applies also to a. Similarly, the magnification bar in d indicates 50 mm, which also applies to c.
Paraneoplastic autonomic neuropathy. Central nervous system sections from a patient with autonomic failure, oat-cell carcinoma of the lung, and positive titer of anti-HU antibodies stained with hematoxylin and eosin (H&E). (a) Inferior olive showing a cluster of mononuclear cells (arrowhead); (b) hippocampus showing perivascular mononuclear infiltrate (arrowhead); (c) midbrain section showing a vessel encased in a mononuclear infiltrate; (d) ventral horn of the thoracic spinal cord showing clusters of mononuclear cells around degenerating motor neurons (arrowheads). Magnification bars indicate 100 mm. The bar in b applies also to a and the bar in d also applies to c.
 
 
 
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