Paraneoplastic cerebellar degeneration associated with serous adenocarcinoma of the ovary

Abstract

We report a case of a 68-year-old woman who presented with symptoms of cerebellar degeneration which initiated a suspicion of underlying malignancy. The patient presented with progressive ataxia and dysarthria and after excluding primary cerebellar pathology, paraneoplastic syndrome was suspected and she was investigated for a malignancy. CT scan of the pelvis showed a left-sided ovarian mass later diagnosed as serous adenocarcinoma of the ovary. She underwent surgery and histology of the mass showed poorly-differentiated serous adenocarcinoma. Paraneoplastic neurological syndrome encompasses several neurological disorders including paraneoplastic cerebellar degeneration (PCD) caused by an immune-mediated mechanism in patients with an underlying malignancy. PCD is a rare condition that occurs in less than 1% of patients with cancer and is associated with specific groups of cancer. It is important to identify PCD due to its association with certain cancers and also to limit the disabilities associated with the syndrome.

Rare case of paraneoplastic cerebellar degeneration secondary to high-grade serous carcinoma of tubo-ovarian origin

“…If no malignancy is found, repeated screening every 3 to 6 months for 2 to 3 years is recommended 14. The necessity of a paraneoplastic antibody profile is debated, due to its lack of sensitivity but high specificity, they are not found in roughly 30% of patients with PNS, and conversely, they can be present in patients without PNS 2 6 11 20 21. Thus, a strong clinical suspicion should be held even if the serology is negative, as in this case.…”

“…As these antibodies are not produced by the tumour and can persist indefinitely, after successful cancer treatment the severe disability continues but no longer progresses 10 12. Although, in multiple cases, tumour resection leads to stabilisation of the neurological disability 6 12 13…”

“…The poor outcome in most, is probably due to how by this stage, neuronal loss is already high and irreversible and immunotherapy is unlikely to affect intrathecal antibodies. Also, anti-Yo alone does not independently cause the condition, as the removal of anti-Yo by plasmapheresis did not improve symptoms,28 29 and animal models injected with anti-Yo immunoglobulin G have failed to reproduce PCD symptoms 6 30. The number of cases is small and new and further studies are needed in order to understand the best treatment option.…”

“…Investigations undertaken for PCD diagnosis are focused towards the search for an underlying tumour, which include chest X-ray, mammogram, CT and pelvic ultrasound 13 15. Onconeural antibodies can be measured but have low sensitivity, so a strong clinical suspicion should not be ignored 2 6 11 20 21…”

“…These include anti-Hu in small cell lung, anti-Ri/Nova in breast and anti-Yo in gynaecological cancers 3. After extensive investigations for a neurological cause of cerebellar syndrome cannot be found, PCD is considered, and full-body imaging,1 4–6 or sometimes a biopsy of a suspicious mass following strong clinical suspicion,3 locates the responsible tumour. In this paper, we report a case of a 69-year-old woman who presented with the cerebellar syndrome and was subsequently diagnosed with PCD as a result of tubo-ovarian carcinoma.…”