Abstract
Paraneoplastic cerebellar degeneration (PCD) is a rare set of neurological disorders arising from tumor-associated autoimmunity against antigens within the cerebellum. Anti-Purkinje cell cytoplasmic antibody 1 (PCA-1), or anti-Yo, is the most commonly linked antibody and is classically associated with breast and ovarian cancers. Here we report case series of PCA-1 associated PCD in patients with known breast or ovarian cancer diagnosis not receiving immunotherapy. These cases highlight recognizing PCA-1 paraneoplastic syndrome triggered by cytotoxic chemotherapy, surgery, tumor recurrence and associated with development of second cancer. Diagnosis of the syndrome requires neurological workup with lumbar puncture (LP) with cerebrospinal fluids (CSF) studies, serum and CSF paraneoplastic antibody panel, and neuroimaging. Inpatient admission for prompt workup and initiation of treatment is recommended. Treatment most commonly includes immunosuppression with corticosteroids, plasmapheresis, and/or intravenous immune globulin (IVIG); however, we postulate that other immune modulating treatments may warrant consideration. In established cancer patients developing this syndrome, workup and treatment of tumor recurrence or development of second malignancy is recommended. These cases highlight the need for early recognition of the syndrome in patients receiving non immune based chemotherapy, for prompt workup and treatment.
Anti-Yo-Associated Paraneoplastic Cerebellar Degeneration: Case Series and Review of Literature
