Journal of Neuro-Oncology

, Volume 81, Issue 1, pp 67–69 | Cite as

Neurologic Improvement after High-dose Intravenous Immunoglobulin Therapy in Patients with Paraneoplastic Cerebellar Degeneration Associated with Anti-Purkinje Cell Antibody

  • Surasak Phuphanich
  • Charles Brock
Clinical – Patient Studies


Paraneoplastic cerebellar degeneration (PCD) is a rare syndrome associated with systemic malignancies, most in lung and ovarian cancer. Cerebellar ataxia has previously been associated with the presence of anti-Purkinje cell antibodies (anti-Yo) in the serum and cerebrospinal fluid and responses to therapy are uncommon. We reported two patients were identified with delayed onset of PCD associated with high titer of CSF anti-Yo (1:30,000, 1:320 U/ml) and a marked elevation of tumor markers for ovarian cancer (CA-125 17,700 ng/ml, 43 ng/ml) titer 1 year and 6 months prior to discovery of the carcinoma. Both developed subacute onset of severe ataxia, dysarthria, tremor, nystagmus with progression to severe debilitation (wheelchair bound or bedridden status). One of these patients also developed dysphagia that required PEG tube feeding. They were treated with six cycles of intravenous immunoglobulin (IVIG) 0.4 gm/kg/day × 5 days, every 4–6 weeks in conjunction with combination chemotherapy of Taxol and Carboplatin after the surgical resection of ovarian cancer. In each case, a significant improvement of neurological deficits were seen after the third cycle of IVIG, approximately 4 months after initiation of treatment. This type of delayed response is contrary to the previous reports. Both patients could ambulate without assistance in correlation with dramatic decrease in anti-Yo titer (1:80, 1:320 U/ml) and CA-125 (11 ng/ml, 8 ng/ml). This is a first report of benefit from IVIG in patients with late onset of PCD, which showed a delayed response with significant neurological improvement.


Ovarian cancer Anti-Yo antibody Cerebellar degeneration Immunoglobulin therapy 


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Surasak Phuphanich, MD was supported in part by a Georgia Cancer Coalition Distinguished Clinical Investigator Professorial Award. We gratefully acknowledge the excellent editing assistance of Ms. Melissa Phuphanich.


  1. 1.
    Ratko TA, Burnett DA, Foulke GE, et al (1995) Recommendations for off-label use of intravenously administered immunoglobulin preparations. JAMA 273:l865–l870CrossRefGoogle Scholar
  2. 2.
    Schwartz SA (1990) Intravenous immunoglobulin for the therapy of autoimmune disorders. J Clinical Immunol 10(2):80–89Google Scholar
  3. 3.
    McCrystal M., Anderson NE, Jones RW, Evans BD (1995) Paraneoplastic cerebellar degeneration in a patient with chemotherapy responsive ovarian cancer. Int J Gynecol Cancer 5:396–399PubMedCrossRefGoogle Scholar
  4. 4.
    Moll JWB, Himden-Loggman SC, Vandur-Mesche FGA, Vescht CHJ (1993) Letter to the Editor: Early diagnosis in intravenous immunoglobulin therapy and paraneoplastic cerebellar degeneration. J Neurol Neurosurg, Psychiatry 56:112CrossRefGoogle Scholar
  5. 5.
    Brower B (1919) Beitrag zur kenntnis der chronischen diffusen kleinhimerkrankunger. Medels Neurol Zentralbatt; 38:674–682Google Scholar
  6. 6.
    Brain R, Wilkinson M (1965) Subacute cerebellar degeneration associated with neoplasms. Brain 8:465–478Google Scholar
  7. 7.
    Anderson NE, Rosenblum MK, Posner JB (1988) Paraneoplastic cerebellar degeneration: clinic and neurologic correlations. Ann Neurol 24:559–567PubMedCrossRefGoogle Scholar
  8. 8.
    Peterson K, Rosenblum MK, Kotanides H, Posner JB (1992) Paraneoplastic cerebellar degeneration. A clinical analysis of 55 anti- Yo antibody-positive patients. Neurology 42:1931–191PubMedGoogle Scholar
  9. 9.
    Hetzel DJ, Stanhope CR, O’Nell VP, Lennon VA (1990) Gynecologic cancer with subacute cerebellar degeneration predicted by Anti-Purkinje cell antibodies in a limited and metastatic volume. Mayo Clin Proc 65(12):1558–1563PubMedGoogle Scholar
  10. 10.
    Paone IF, Jeyasingham KJ (1980) Remission of cerebellar dysfunction of pneumonectomy for bronchiogenic carcinoma. N Engl J Med 3:156CrossRefGoogle Scholar
  11. 11.
    Rewcastle NB (1963) Subacute cerebellar degeneration with Hodgkin’s disease. Arch Neurology 9:407–413Google Scholar
  12. 12.
    Fumeaux HM, Rosenblum MK, Dolmer J (1990) Selective expression of Purkinje-cell antigens in tumor tissue from paraneoplastic degeneration. N Engl J Med 322: 1844–1851CrossRefGoogle Scholar
  13. 13.
    Cocconi G, Ceri G, Juvorra G, Alt ET (1985) Successful treatment of subacute cerebellar degeneration in ovarian carcinoma with plasmapheresis. Cancer 56:2318–2320PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media B.V. 2006

Authors and Affiliations

  1. 1.Neuro-Oncology Program, Winship Cancer Institute, Department of Hematology-Oncology Emory University School of MedicineAtlantaUSA
  2. 2.Department of NeurologyUniversity of South FloridaTampaUSA

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