Journal of Neuro-Oncology

, Volume 63, Issue 2, pp 187–190 | Cite as

Response to intravenous immunoglobulin in Anti-Yo associated paraneoplastic cerebellar degeneration: case report and review of the literature

  • Peter Widdess-Walsh
  • Jinny O. Tavee
  • Stephan Schuele
  • Glen H. Stevens


Paraneoplastic cerebellar degeneration (PCD) is a debilitating neuro-degenerative disease associated with antibodies directed against the purkinje cells of the cerebellum. Treatment using chemotherapy or other treatment of the primary tumor to various immunologically directed therapies has been attempted but outcomes have been poor. We discuss a patient with ovarian carcinoma and PCD seen in our institution who showed a marked beneficial response to intravenous immunoglobulin (IVIG) and methylprednisolone. A Medline search from 1966-2002 produced fifteen cases of PCD confirmed by antibody testing that were treated with IVIG, either alone, or with a combination of other therapies. The clinical characteristics and treatment responses of these patients are analyzed in this review. Most patients that were treated with IVIG and had what was defined as a good response were treated within one month of symptoms. Patients treated between one month and three months often had stable disease and patients treated after three months of symptoms usually had a poor outcome. Early treatment with sufficiently high doses of IVIG seems to provide a better chance of treatment success. The additional benefit of early high dose intravenous methylprednisolone is unclear. Due to the devastating nature of the disease, a trial of IVIG and steroids is warranted as early as possible in a dose of 2g/kg to any patient with a clinical picture of PCD and positive antibodies.

cerebellar immunoglobulin IVIG methylprednisolone ovarian paraneoplastic 


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  1. 1.
    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 47: 9-17, 2000Google Scholar
  2. 2.
    Rojas I, Graus F, Keime-Guibert F, Rene R, Delattre JY, Ramon JM, Dalmau J, Posner JB: Long-term clinical outcome of paraneoplastic cerebellar degeneration and anti-Yo antibodies. Neurology 55(5): 713-715, 2000Google Scholar
  3. 3.
    Counsell CE, McLeod M, Grant R: Reversal of subacute paraneoplastic cerebellar syndrome with intravenous immunoglobulin. Neurology 44: 1184-1185, 1994Google Scholar
  4. 4.
    Moll JWB, Henzen-Logmans SC, Van der Meche FGA, Vecht Ch J: Early diagnosis and intravenous immunoglobulin therapy in paraneoplastic cerebellar degeneration. J Neurol Neurosurg Psychiatry 62: S73-S81, 1993Google Scholar
  5. 5.
    Uchuya M, Graus F, Vega F, Rene R, Delattre JY: Intravenous immunoglobulin treatment in paraneoplastic neurological syndromes with antineuronal autoantibodies. J Neurol Neurosurg Psychiatry 60(4): 388-392, 1996Google Scholar
  6. 6.
    Blaes F, Strittmatter M, Merkelbach S, Jost V, Klotz M, Schimrigk K, Hamann GF: Intravenous immunoglobulins in the therapy of paraneoplastic neurological disorders. J Neurol 246(4): 299-303, 1999Google Scholar
  7. 7.
    David YB, Warner E, Levitan M, Sutton DM, Malkin MG, Dalmau JO: Autoimmune paraneoplastic cerebellar degeneration in ovarian carcinoma patients treated with plasmapheresis and immunoglobulin. A case report. Cancer 78(10): 2153-2156, 1996Google Scholar
  8. 8.
    Graus F, Vega F, Delattre JY, Bonaventura I, Rene R, Arbaiza D, Tolosa E: Plasmapheresis and antineoplastic treatment in CNS paraneoplastic syndromes with antineuronal autoantibodies. Neurology 42(3 Pt 1): 536-540, 1992Google Scholar
  9. 9.
    Keime-Guibert F, Graus F, Fleury A, Rene R, Honnorat J, Broet P, Delattre JY: Treatment of paraneoplastic neurological syndromes with antineuronal antibodies (Anti-Hu, anti-Yo) with a combination of immunoglobulins, cyclophosphamide, and methylprednisolone. J Neurol Neurosurg Psychiatry 68(4): 479-482, 2000Google Scholar

Copyright information

© Kluwer Academic Publishers 2003

Authors and Affiliations

  • Peter Widdess-Walsh
    • 1
  • Jinny O. Tavee
    • 1
  • Stephan Schuele
    • 1
  • Glen H. Stevens
    • 1
  1. 1.Department of NeurologyCleveland Clinic FoundationClevelandUSA

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