eMedicine Specialties > Neurology > Neuro-oncology
Paraneoplastic Cerebellar Degeneration: Treatment & Medication
Updated: Jul 13, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Two approaches can be used to treat paraneoplastic neurologic syndrome. The first treatment is directed toward the underlying tumor, while the second approach is toward the autoimmune disease causing the cerebellar dysfunction.
Since neurologic paraneoplastic syndromes are immune-mediated, 2 distinct approaches to therapy have been reported: removal of the antigen source by treatment of the underlying tumors and suppression of the immune response. Immunosuppression can be beneficial for some conditions.20
- Paraneoplastic syndromes are a therapeutic challenge for the neurologist, and treatment of paraneoplastic syndromes is generally unsatisfactory.
- Early tumor detection and treatment should be the primary objective in these patients.
- The response of the paraneoplastic neurologic syndromes to immunosuppressive agents or antitumor treatment is influenced greatly by the underlying neuropathology.
- The effect of the combination of intravenous immunoglobulins (IVIG), cyclophosphamide, and methylprednisolone on the clinical course of patients with paraneoplastic neurologic syndrome or paraneoplastic cerebellar degeneration and antineuronal antibodies is unsatisfactory.
- Some reports indicate partial or complete remission of cerebellar symptoms after treating the primary neoplasm. This has been observed only in small-cell carcinomas and is not reported in gynecologic malignancies.
- In a minority of patients who are not disabled severely at the onset of treatment, a transient stabilization is possible and deserves further evaluation.
Surgical Care
Surgical care is required for patients who undergo tumor resection.
Consultations
A team approach is required in treating patients with paraneoplastic cerebellar degeneration.
- Neurologic consultation is needed for basic workup and to exclude other possible causes of cerebellar dysfunction.
- Oncology consultation is needed for tumor workup and treatment protocols.
- Surgical consultation is needed in patients for whom tumor resection is recommended.
Diet
The patient may require nutritional support in severe cases of nausea and vomiting.
Activity
Bed rest is usual because patients with severe cerebellar dysfunction are at high risk of falls.
More on Paraneoplastic Cerebellar Degeneration |
| Overview: Paraneoplastic Cerebellar Degeneration |
| Differential Diagnoses & Workup: Paraneoplastic Cerebellar Degeneration |
Treatment & Medication: Paraneoplastic Cerebellar Degeneration |
| Follow-up: Paraneoplastic Cerebellar Degeneration |
| Multimedia: Paraneoplastic Cerebellar Degeneration |
| References |
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References
Lorusso L, Hart IK, Giometto B, et al. Immunological features of neurological paraneoplastic syndromes. Int J Immunopathol Pharmacol. May-Aug 2004;17(2):135-44. [Medline].
BRAIN WR, DANIEL PM, GREENFIELD JG. Subacute cortical cerebellar degeneration and its relation to carcinoma. J Neurol Neurosurg Psychiatry. May 1951;14(2):59-75. [Medline].
Posner JB. Paraneoplastic cerebellar degeneration. Can J Neurol Sci. May 1993;20 Suppl 3:S117-22. [Medline].
Inuzuka T. Autoantibodies in paraneoplastic neurological syndrome. Am J Med Sci. Apr 2000;319(4):217-26. [Medline].
Bolla L, Palmer RM. Paraneoplastic cerebellar degeneration. Case report and literature review. Arch Intern Med. Jun 9 1997;157(11):1258-62. [Medline].
Nath U, Grant R. Neurological paraneoplastic syndromes. J Clin Pathol. Dec 1997;50(12):975-80. [Medline].
Albert ML, Austin LM, Darnell RB. Detection and treatment of activated T cells in the cerebrospinal fluid of patients with paraneoplastic cerebellar degeneration. Ann Neurol. Jan 2000;47(1):9-17. [Medline].
Okano HJ, Park WY, Corradi JP, Darnell RB. The cytoplasmic Purkinje onconeural antigen cdr2 down-regulates c-Myc function: implications for neuronal and tumor cell survival. Genes Dev. Aug 15 1999;13(16):2087-97. [Medline].
Rojas I, Graus F, Keime-Guibert F, et al. Long-term clinical outcome of paraneoplastic cerebellar degeneration and anti-Yo antibodies. Neurology. Sep 12 2000;55(5):713-5. [Medline].
Schmahmann JD, Sherman JC. The cerebellar cognitive affective syndrome. Brain. Apr 1998;121 ( Pt 4):561-79. [Medline].
Peterson K, Rosenblum MK, Kotanides H, Posner JB. Paraneoplastic cerebellar degeneration. I. A clinical analysis of 55 anti-Yo antibody-positive patients. Neurology. Oct 1992;42(10):1931-7. [Medline].
Tanaka M, Tanaka K, Shinozawa K, et al. Cytotoxic T cells react with recombinant Yo protein from a patient with paraneoplastic cerebellar degeneration and anti-Yo antibody. J Neurol Sci. Nov 26 1998;161(1):88-90. [Medline].
Greenlee JE, Brashear HR. Antibodies to cerebellar Purkinje cells in patients with paraneoplastic cerebellar degeneration and ovarian carcinoma. Ann Neurol. Dec 1983;14(6):609-13. [Medline].
Jaeckle KA, Graus F, Houghton A. Autoimmune response of patients with paraneoplastic cerebellar degeneration to a Purkinje cell cytoplasmic protein antigen. Ann Neurol. Nov 1985;18(5):592-600. [Medline].
Mason WP, Graus F, Lang B, Honnorat J, Delattre JY, Valldeoriola F. Small-cell lung cancer, paraneoplastic cerebellar degeneration and the Lambert-Eaton myasthenic syndrome. Brain. Aug 1997;120 ( Pt 8):1279-300. [Medline].
Wilkinson PC, Zeromski J. Immunofluorescent detection of antibodies against neurones in sensory carcinomatous neuropathy. Brain. Sep 1965;88(3):529-83. [Medline].
Graus F, Cordon-Cardo C, Posner JB. Neuronal antinuclear antibody in sensory neuronopathy from lung cancer. Neurology. Apr 1985;35(4):538-43. [Medline].
Graus F, Elkon KB, Cordon-Cardo C, Posner JB. Sensory neuronopathy and small cell lung cancer. Antineuronal antibody that also reacts with the tumor. Am J Med. Jan 1986;80(1):45-52. [Medline].
Dalmau J, Gonzalez RG, Lerwill MF. Case records of the Massachusetts General Hospital. Case 4-2007. A 56-year-old woman with rapidly progressive vertigo and ataxia. N Engl J Med. Feb 8 2007;356(6):612-20. [Medline].
Rosenfeld MR, Dalmau J. Current Therapies for Paraneoplastic Neurologic Syndromes. Curr Treat Options Neurol. Jan 2003;5(1):69-77. [Medline].
Bataller L, Dalmau J. Paraneoplastic neurologic syndromes: approaches to diagnosis and treatment. Semin Neurol. Jun 2003;23(2):215-24. [Medline].
Dalmau JO, Posner JB. Paraneoplastic syndromes. Arch Neurol. Apr 1999;56(4):405-8. [Medline].
Darnell RB. The importance of defining the paraneoplastic neurologic disorders. N Engl J Med. Jun 10 1999;340(23):1831-3. [Medline].
Darnell RB, Posner JB. Paraneoplastic syndromes involving the nervous system. N Engl J Med. Oct 16 2003;349(16):1543-54. [Medline].
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].
Greenlee JE. Cytotoxic T cells in paraneoplastic cerebellar degeneration. Ann Neurol. Jan 2000;47(1):4-5. [Medline].
Greenlee JE, Brashear HR, Jaeckle KA, et al. Pursuing an occult carcinoma in a patient with subacute cerebellar degeneration and anticerebellar antibodies. Need for vigorous follow-up. West J Med. Feb 1992;156(2):199-202. [Medline].
Jaeckle KA. Paraneoplastic nervous system syndromes. Curr Opin Oncol. May 1996;8(3):204-8. [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].
Rousseau A, Benyahia B, Dalmau J, Connan F, Guillet JG, Delattre JY, et al. T cell response to Hu-D peptides in patients with anti-Hu syndrome. J Neurooncol. Feb 2005;71(3):231-6. [Medline].
Sommer C, Weishaupt A, Brinkhoff J, Biko L, Wessig C, Gold R. Paraneoplastic stiff-person syndrome: passive transfer to rats by means of IgG antibodies to amphiphysin. Lancet. Apr 16-22 2005;365(9468):1406-11. [Medline].
Voltz RD, Posner JB, Dalmau J, Graus F. Paraneoplastic encephalomyelitis: an update of the effects of the anti- Hu immune response on the nervous system and tumour. J Neurol Neurosurg Psychiatry. Aug 1997;63(2):133-6. [Medline].
Further Reading
Keywords
paraneoplastic neurologic syndrome, PCD, breast cancer, ovarian cancer, uterine cancer, lung cancer, occult gynecologic cancers, malignancy, paraneoplastic cerebellar degeneration
Treatment & Medication: Paraneoplastic Cerebellar Degeneration