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. Medical records of patients at our institution who developed PCA-1 associated PCD were reviewed. Clinical information, including cancer history, cancer-directed treatment, and serum and CSF titers of PCA-1 antibody were extracted. We report a series of cases of PCA-1 associated PCD in patients with known breast or ovarian cancer diagnosis not receiving immunotherapy. These cases highlight aspects of PCA-1 paraneoplastic syndrome such as triggering by cytotoxic chemotherapy or surgery, the possibility of tumor recurrence and the association with development of a 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. These cases highlight the need for early recognition of the syndrome in patients receiving nonimmune based chemotherapy, for prompt workup and treatment.
Highlights
Paraneoplastic cerebellar degeneration (PCD) is a rare set of neurological disorders arising from autoantibodies against host antigens within the cerebellum in the setting of cancer
Paraneoplastic cerebellar degeneration (PCD) is a rare set of neurological disorders arising from tumorassociated autoimmunity against antigens within the cerebellum
We report case series of Purkinje cell cytoplasmic antibody 1 (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
Summary
Paraneoplastic cerebellar degeneration (PCD) is a rare set of neurological disorders arising from autoantibodies against host antigens within the cerebellum in the setting of cancer. The clinical syndrome of PCD usually precedes a cancer diagnosis, with one study estimating that 63% of patients are diagnosed with a malignancy during the evaluation of their neurological symptoms.[10,12,13] some tumors are not detectable when the syndrome is diagnosed, and expert clinicians have recommend thorough cancer surveillance with routine age-appropriate screening plus whole-body imaging every 3 to 6 months for 2 to 3 years following PCD diagnosis.[12,13] some patients will have a prolonged subclinical course, with one study where patients who screened positive on serum antibody tests shortly after the diagnosis of cancer developing symptoms of up to 5 years later.[12] In 30% of PCD cases, ataxia develops during a period of cancer remission.[12,14] Given the variable timing of presentation, providers should maintain clinical suspicion for PCD regardless of the neoplastic disease course. These cases highlight the importance of early recognition and treatment of PCA-1 paraneoplastic syndrome and its possible indication of a new or recurrent cancer diagnosis
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