eMedicine Specialties > Neurology > Neuro-oncology

Paraneoplastic Autonomic Neuropathy: Follow-up

Author: Bjorn E Oskarsson, MD, Assistant Professor, Department of Neurology, University of California Davis
Coauthor(s): Dianna Quan, MD, Associate Professor of Neurology, Director, Electromyography Laboratory, University of Colorado Health Sciences Center; Ronald G Wiley, MD, PhD, Chief, Professor, Department of Neurology, Veterans Affairs Medical Center, Vanderbilt University
Contributor Information and Disclosures

Updated: Jul 12, 2006

Follow-up

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.

Further Outpatient Care

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

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.

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.

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.

Prognosis

  • Prognosis is 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 years18 . The major exception is patients with Lambert-Eaton syndrome, in whom effective treatment may result in remission of the often mild autonomic symptoms and better than expected survival24,25,26 .

Miscellaneous

Medicolegal Pitfalls

  • One issue that can arise is failure to detect the associated malignancy at the time of presentation of autonomic failure. Repeated investigation for an underlying cancer is indicated. However, tumors can elude detection by all known diagnostic means for months or years in some patients, in spite of vigorous attempts at detecting the tumor.

Special Concerns

  • Since the presumed pathogenesis is an idiosyncratic autoimmune response, these patients are not contagious and their management is directed primarily at the associated malignancy and management of autonomic symptoms.
 


More on Paraneoplastic Autonomic Neuropathy

Overview: Paraneoplastic Autonomic Neuropathy
Differential Diagnoses & Workup: Paraneoplastic Autonomic Neuropathy
Treatment & Medication: Paraneoplastic Autonomic Neuropathy
Follow-up: Paraneoplastic Autonomic Neuropathy
Multimedia: Paraneoplastic Autonomic Neuropathy
References
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References

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  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].

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  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].

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  7. 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].

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Further Reading

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Keywords

anti-HU disease paraneoplastic encephalomyeloneuropathy, autonomic dysfunction, paraneoplastic neurological degeneration, paraneoplastic autonomic neuropathy, PNS, paraneoplastic syndromes, autonomic neuropathy, paraneoplastic peripheral neuropathies, paraneoplastic encephalomyeloneuropathies, Lambert-Eaton myasthenic syndrome, LEMS

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Contributor Information and Disclosures

Author

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.

Coauthor(s)

Dianna Quan, MD, Associate Professor of Neurology, Director, Electromyography Laboratory, University of Colorado Health Sciences Center
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.

Medical Editor

Amy A Pruitt, MD, Associate Professor of Neurology, University of Pennsylvania; 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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
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: Nothing to disclose.

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  Honoraria Consulting; Teva Marion Honoraria Consulting; Boeringer-Ingelheim Honoraria Speaking and teaching

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