Article Text
Abstract
Introduction/Background Paraneoplastic cerebellar degeneration (PCD) is a subtype of paraneoplastic syndromes (PNS) associated with ovarian malignancy. PCD presents with subacute cerebellar ataxia and dysarthria secondary to loss of purkinje cells1. PCD is rare and considered a diagnosis of exclusion. Despite its rarity, it’s an important area of research due the significant impact on quality of life for patients with PCD3. Here we present a case report and review of the literature.
Case report A 49-year-old women presented with bilateral paraesthesia in her hands progressing over a 6-month period culminating with the patient being rendered bedbound with significant dysarthria. Investigations including positive anti-YO antibodies in cerebrospinal fluid sampling, adnexal mass, and raised CA125 led to PCD being diagnosed. The patient underwent staging surgery 7 months after initial symptom onset. Histological diagnosis confirmed FIGO Stage 3B high-grade serous ovarian cancer. Despite surgical resection she remains bedbound with ongoing speech difficulties.
Methodology A scoping review of the literature on PCD and ovarian malignancy was conducted. Medline database and Pubmed were used. The literature was then reviewed, with common themes identified and reported.
Results The rarity of PCD limits the quality of evidence published to expert opinion, case reports/series. Presentation differs depending on associated antibodies identified. Anti-YO antibodies are almost exclusively associated with ovarian cancer and are present in 50% of ovarian-cancer-associated PCD. Anti-YO antibody is associated with severe incapacitation. A variety of adjunctive treatments such as plasma exchange have been suggested in anti-YO positive PCD with limited efficacy/evidence to support use.
Conclusion PNS are a rare but important complication of ovarian malignancy. PCD can be severely incapacitating and have a significant impact on patient’s quality of life after surgery. Current literature is limited due condition’s rarity. Limiting delay between symptom onset and surgical management would reduce long term morbidity.
Disclosures No disclosures.