Objective: Paraneoplastic cerebellar degeneration (PCD) is a rare neurological complication of cancer characterized by rapid development of cerebellar ataxia resulting from tumor-induced autoimmunity against cerebellar purkinje cells. Anti-Yo antibody which is also known as anti-Purkinje cell cytoplasmic antibody type-1, is highly specific and the most frequent antibody in patients with PCD. Here we present a case of anti-Yo-associated PCD in a patient with ovarian cancer. After the patient was diagnosed with PCD, ovarian cancer recurrence was shown.
Case report: A 54-year-old female patient, who was in remission with ovarian cancer applied to us with a 6-month history of progressively worsening unsteadiness while walking. She was diagnosed as ovarian cancer in November 2016 and operated, and then 6 cycles of carboplatin plus paclitaxel adjuvant treatment was given. She did not have any other disease and history of drug, smoking, and alcohol use. There was no important family history. On physical examination, her speech was minimally dysarthric. While she was walking, ataxia was observed. Other system examinations were normal. Hemogram, biochemistry, muscle enzymes, thyroid function tests, vitamin B12, and 25-OH-D were in the normal range. CA-125 increased compared to 3 months ago.(23–53U/mL)Because of the increased CA-125 level, computer tomography and then PET-CT scan was taken. There was a 1.5-cm diameter hypermetabolic nodular pelvic lesion. Brain MR and EMG were planned for complaints of walking and balance disorders. Nothing was found in the examinations and tests to explain the current condition of the patient. The paraneoplastic panel was taken from the blood and cerebrospinal fluid (CSF) samples. Anti-Yo antibodies were three positive in both the CSF and blood samples. The patient was diagnosed with PCD due to clinical findings and anti-Yo positivity both CSF and blood samples. Since the main treatment of paraneoplastic syndrome was the excision of the primary lesion, it was discussed for the excision of the recurrent mass. But this patient was not eligible for re-surgery. So carboplatin, gemcitabine plus bevacizumab treatment protocol was initiated for recurrent ovarian cancer. Plasmapheresis was performed 5 times, every other day. A significant improvement in walking were observed in the patient after 2 weeks from discharge.
Conclusion: Here we described a patient who developed ataxia 3 years after remission of ovarian cancer and diagnosed with PCD. Diagnosing a paraneoplastic syndrome and mild elevation of CA-125 level have led to the diagnosis of recurrence of ovarian cancer. In approximately 30% of patients, the ataxic symptoms occur when the cancer is in remission as it was in this reported case. Therefore, when a patient is diagnosed with PCD, whole-body screening is necessary to reveal the underlying malignancy. Although there is a strong association between PCD and Anti-yo; its pathological function is still not clear. Treatment of PCD is unfavorable and patients usually have a poor prognosis. Plasmapheresis, intravenous immunoglobulin (IVIG), and cyclophosphamide are the treatment options. Also, it is very important to treat underlying malignancy. In conclusion, in patients with unexplained neurological symptoms and a history of cancer, paraneoplastic syndromes should be considered and an underlying malignancy should be investigated.
