title: Neurologic improvement after high-dose intravenous immunoglobulin therapy in patients with paraneoplastic cerebellar degeneration associated with anti-Purkinje cell antibody.
abstract: 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 x 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.
authors.affiliation: Neuro-Oncology Program, Winship Cancer Institute, Department of Hematology-Oncology , Emory University School of Medicine, 1365 Clifton Road, NE, Room C-5002, Atlanta, GA 30322, USA. surasak.phuphanich@emoryhealthcare.org
authors.forename: Surasak
authors.initials: S
authors.lastname: Phuphanich
authors.forename: Charles
authors.initials: C
authors.lastname: Brock
mesh_terms: D000368:Aged; D002277:Carcinoma / Q000150:complications / Q000276:immunology* / Q000473:pathology; D004305:Dose-Response Relationship, Drug; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D008875:Middle Aged; D010051:Ovarian Neoplasms / Q000150:complications / Q000276:immunology* / Q000473:pathology; D020362:Paraneoplastic Cerebellar Degeneration / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D010534:Peritoneal Neoplasms / Q000150:complications / Q000276:immunology / Q000473:pathology; D011689:Purkinje Cells / Q000276:immunology* / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors