A 54-Year-Old Woman with Progressive Ataxia

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Kun Huang MD PhD
Robin Parker MD FRCPC
Tabassum Firoz MD FRCPC


Paraneoplastic cerebellar degeneration (PCD) is a rare but devastating syndrome associated with autoantibodies targeting cerebellar antigens expressed by tumours outside of nervous system. PCD is most often associated with breast and gynecological cancers and can precede clinical manifestations of the cancer. Of the dozen autoantibodies associated with PCD, anti-Yo is the most commonly identified. In this report, we present a case of a woman with progressive cerebellar ataxia and anti-Yo antibody, who has microscopic high-grade serous carcinoma found with empiric total abdominal hysterectomy bilateral salpingo-oophorectomy (TAH-BSO). This case highlights the challenges in diagnosis, difficulty in identifying the occult malignancy, and the need for multidisciplinary collaboration between Internal Medicine, Neurology, Gynecology and Pathology. The literature relating to diagnosis, prognosis and treatment of PCD is also reviewed.
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1. Szubert S, Michalak S, Szpurek D, Moszynski R, Krygowska-Zielinska J, Sajdak S. Anti-ovarian antibodies in sera of patients with ovarian tumors. Immunol Lett. 2012;148(2):133-137. doi:10.1016/j.imlet.2012.09.008.
2. Rojas I, Graus F, Keime-Guibert F, et al. Long-term clinical outcome of paraneoplastic cerebellar degeneration and anti-Yo antibodies. Neurology. 2000;55(5):713-715. http://www.ncbi.nlm.nih.gov/pubmed/10980743.
3. Bradley WH, Dottino PR, Rahaman J. Paraneoplastic cerebellar degeneration in ovarian carcinoma: case report with review of immune modulation. Int J Gynecol Cancer. 2008;18(6):1364-1367. doi:10.1111/j.1525- 1438.2007.01173.x.
4. Peterson K, Rosenblum MK, Kotanides H, Posner JB. Paraneoplastic cerebellar degeneration. I. A clinical analysis of 55 anti-Yo antibody-positive patients. Neurology. 1992;42(10):1931-1937. http://www.ncbi.nlm.nih.gov/ pubmed/1407575.
5. Bernal F, Shams’ili S, Rojas I, et al. Anti-Tr antibodies as markers of paraneoplastic cerebellar degeneration and Hodgkin’s disease. Neurology. 2003;60(2):230-234. http://www.ncbi.nlm.nih.gov/pubmed/12552036.
6. Graus F, Delattre JY, Antoine JC, et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry. 2004;75(8):1135-1140. doi:10.1136/jnnp.2003.034447.
7. Hetzel DJ, Stanhope CR, O’Neill BP, Lennon VA. Gynecologic cancer in patients with subacute cerebellar degeneration predicted by anti- Purkinje cell antibodies and limited in metastatic volume. Mayo Clin Proc. 1990;65(12):1558-1563. http://www.ncbi.nlm.nih.gov/pubmed/2255217.
8. Blaes F, Strittmatter M, Merkelbach S, et al. Intravenous immunoglobulins in the therapy of paraneoplastic neurological disorders. J Neurol. 1999;246(4):299-303. http://www.ncbi.nlm.nih.gov/pubmed/10367699.
9. Shams’ili S, de Beukelaar J, Gratama JW, et al. An uncontrolled trial of rituximab for antibody associated paraneoplastic neurological syndromes. J Neurol. 2006;253(1):16-20. doi:10.1007/s00415-005-0882-0.
10. Giometto B, Grisold W, Vitaliani R, et al. Paraneoplastic neurologic syndrome in the PNS Euronetwork database: a European study from 20 centers. Arch Neurol. 2010;67(3):330-335. doi:10.1001/archneurol.2009.341.
11. Shams’ili S, Grefkens J, de Leeuw B, et al. Paraneoplastic cerebellar degeneration associated with antineuronal antibodies: analysis of 50 patients. Brain. 2003;126(Pt 6):1409-1418. http://www.ncbi.nlm.nih.gov/ pubmed/12764061.
12. Honnorat J, Cartalat-Carel S. Advances in paraneoplastic neurological syndromes. Curr Opin Oncol. 2004;16(6):614-620. doi:00001622-200411000- 00017 [pii].