Isolated ZIC4 antibodies in paraneoplastic cerebellar syndrome with an underlying ovarian tumor.

OBJECTIVE To describe a patient with paraneoplastic cerebellar syndrome and the uncommon association of isolated ZIC4 antibodies and ovarian cancer. DESIGN Case report and review of the literature. SETTING Hospitalized care, follow-up in private practice. PATIENT A 60-year-old woman with severe paraneoplastic cerebellar syndrome and an underlying ovarian adenocarcinoma. INTERVENTIONS Neurological examination, lumbar puncture, laboratory tests, radiological imaging, and histological examination. MAIN OUTCOME MEASURES Clinical course and titer of anti- ZIC4 antibodies in serum. RESULTS Laboratory and cerebrospinal fluid tests revealed the isolated presence of ZIC4 antibodies. Screening results for small cell lung carcinoma were negative, while abdominal computed tomographic scan was suggestive of ovarian adenocarcinoma, which was confirmed by histological examination. Glucocorticosteroid administration and chemotherapy led to complete remission of paraneoplastic cerebellar degeneration. CONCLUSION To the best of our knowledge, this is the first case of paraneoplastic cerebellar degeneration in a patient with isolated ZIC4 antibodies associated with ovarian adenocarcinoma.