Autoimmune paraneoplastic cerebellar degeneration in ovarian carcinoma patients treated with plasmapheresis and immunoglobulin. A case report.

BACKGROUND

Paraneoplastic cerebellar degeneration (PCD) is a remote effect of cancer most frequently associated with carcinoma of the ovary or lung. In many patients, antibodies to Purkinje cells are found. Progressive, incapacitating cerebellar dysfunction occurs in most cases, and no treatment has produced even a transient response in any significant proportion of patients.

METHODS

A woman age 81 years with recurrent ovarian carcinoma and PCD, confirmed clinically, radiologically, and serologically, was treated with 5 exchanges of 1 plasma volume each, followed by intravenous immunoglobulin at a dose of 1g/Kg-1 body weight daily for 2 days.

RESULTS

Several weeks after the treatment, the patient had significant improvement of her dizziness, tremor, and dysmetria. She refused maintenance therapy and began to deteriorate neurologically 3 months after the treatment.

CONCLUSIONS

Although this is only a single case report, the authors believe that the dire prognosis of PCD and the lack of effective therapy warrant a trial of this combined treatment early in the course of the disease. Confirmatory evidence of the efficacy of such an approach would be welcomed.