eMedicine Specialties > Neurology > Neuro-oncology
Paraneoplastic Autonomic Neuropathy: Treatment & Medication
Updated: Jul 12, 2006
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Care of patients with paraneoplastic autonomic neuropathy depends on severity of autonomic failure and status of associated malignancy.
- Malignancy
- The most important aspect of medical care in a patient with paraneoplastic autonomic dysfunction is treatment of the underlying malignancy. If the malignancy can be cured, progression of autonomic dysfunction may cease and potentially even reverse. If a cure is not possible and chemotherapy is a consideration, then a nonneurotoxic regimen such as cyclophosphamide and doxorubicin (Adriamycin) that is immunosuppressive is theoretically appealing. However, no data show superiority of any particular regimen, as long as the tumor is controlled.
- In exceptional cases, patients presenting with typical encephalomyeloneuropathy, including autonomic failure and positive antineuronal antibody titers, may be considered for chemotherapy even in the absence of a tissue diagnosis. The risk of an occult malignancy in this specific situation is very high.
- Autoimmune process: In general, immunosuppressive treatments directed against the paraneoplastic antibodies (eg, plasmapheresis) have been disappointing, but occasional patients have had benefits23 . No therapy directed at suppressing the immune system has been shown to be reliably effective; however, successful treatment of the associated malignancy by resection and/or chemotherapy may slow or stop the progression of the neurological syndrome.
- Autonomic failure
- Nonpharmacologic measures are useful for all patients with autonomic dysfunction:
- Discontinue antihypertensive medications and other medications known to lower blood pressure, if feasible.
- Increase fluid and salt intake.
- Equipment aids may be helpful; these include tight support stockings, abdominal binders or antigravity suits for symptomatic hypotension, and bladder catheterization for urinary retention.
- Dietary fiber and enemas may help improve bowel motility and decrease straining during defecation.
- Patients with decreased sweating should limit their physical activity, particularly in hot weather. Sponging with water during activity may help prevent overheating.
- Large meals may exacerbate hypotension and should be avoided.
- Positional changes, such as standing, should be performed slowly and gradually.
- Elevate the head of the bed and avoid prolonged recumbency.
- Many pharmacological interventions directed against symptoms of autonomic failure are helpful; please see the article on Idiopathic Orthostatic Hypotension and Other Autonomic Failure Syndromes for a discussion.
- Nonpharmacologic measures are useful for all patients with autonomic dysfunction:
Consultations
If no known underlying cancer is present, consider seeking consultation from a pulmonary medicine specialist. A pulmonologist can help in finding small cell lung cancer or other lung tumor through imaging and bronchoscopy. A medical oncology specialist can be of diagnostic help, in addition to assisting in the treatment of the associated malignancy.
Gastroenterology and urology consultation may be needed if intestinal dysmotility and urinary incontinence are part of the clinical manifestations.
Diet
High fluid and salt intake is of potential value for orthostatic hypotension. A fiber rich diet can help some gastric dysmotility symptoms.
Medication
No specific drug treatment is of proven value; however, cytotoxic chemotherapy for the associated malignancy is rational. For symptomatic management of autonomic failure, see article on Idiopathic Orthostatic Hypotension and Other Autonomic Failure Syndromes for details.
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 |
| « Previous Page | Next Page » |
References
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].
Anderson NE, Rosenblum MK, Graus F. Autoantibodies in paraneoplastic syndromes associated with small-cell lung cancer. Neurology. Sep 1988;38(9):1391-8. [Medline].
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].
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].
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].
Ficker DM, Hammack JE. Primary central nervous system lymphoma presenting as autonomic dysfunction. J Neurooncol. 1995;23(3):245-8. [Medline].
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].
Pittock SJ, Kryzer TJ, Lennon VA. Paraneoplastic antibodies coexist and predict cancer, not neurological syndrome. Ann Neurol. Nov 2004;56(5):715-9. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
Chinn JS, Schuffler MD. Paraneoplastic visceral neuropathy as a cause of severe gastrointestinal motor dysfunction. Gastroenterology. Nov 1988;95(5):1279-86. [Medline].
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].
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].
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].
Khurana RK, Koski CL, Mayer RF. Autonomic dysfunction in Lambert-Eaton myasthenic syndrome. J Neurol Sci. May 1988;85(1):77-86. [Medline].
Maddison P, Lang B. Paraneoplastic neurological autoimmunity and survival in small-cell lung cancer. J Neuroimmunol. Sep 15 2008;201-202:159-62. [Medline].
Mamdani MB, Walsh RL, Rubino FA. Autonomic dysfunction and Eaton Lambert syndrome. J Auton Nerv Syst. Apr 1985;12(4):315-20. [Medline].
Further Reading
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
Treatment & Medication: Paraneoplastic Autonomic Neuropathy