Access the full text.
Sign up today, get DeepDyve free for 14 days.
Hindawi Case Reports in Oncological Medicine Volume 2020, Article ID 9021843, 3 pages https://doi.org/10.1155/2020/9021843 Case Report Combined Cerebellar and Spinal Cord Deficits Caused by an Underlying Gynecologic Malignancy 1 2 3 4 Tamer Othman , Moshe-Samuel Hendizadeh, Ritika Vankina, Susan Park , and Phyllis Kim Department of Internal Medicine, Harbor-UCLA Medical Center, Torrance, CA, USA Department of Neurology, Harbor-UCLA Medical Center, Torrance, CA, USA Division of Hematology and Medical Oncology, Harbor-UCLA Medical Center, Torrance, CA, USA Division of Gynecology Oncology, Harbor-UCLA Medical Center, Torrance, CA, USA Department of Hematology and Oncology, Kaiser Permanente Los Angeles Medical Center, Los Angeles, CA, USA Correspondence should be addressed to Tamer Othman; email@example.com Received 31 March 2019; Revised 25 December 2019; Accepted 2 January 2020; Published 9 January 2020 Academic Editor: Raﬀaele Palmirotta Copyright © 2020 Tamer Othman et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Paraneoplastic cerebellar degeneration (PCD) is an uncommon autoimmune disorder targeting antigens within the nervous system and is usually associated with an underlying malignancy. Neurologic symptoms frequently precede the cancer diagnosis, which is most often seen in women with breast or gynecologic tumors. Anti-Yo-related PCD is the most common PCD syndrome, and one of the best understood. Although cerebellar signs are characteristic of anti-Yo PCD, myelopathy is an unusual presentation of anti- Yo PCD based on published case series and reports. Unfortunately, the prognosis for anti-Yo PCD is often poor, and most patients become bedridden. We report a case highlighting a severe presentation of cerebellar degeneration along with an unusual ﬁnding of myelopathy in a patient with a newly diagnosed gynecologic cancer. as well as myelopathy, in a patient that was found to have 1. Introduction positive anti-Yo titers and a gynecologic tumor, which is PCD results from autoantibodies produced by tumors that not typical of this disorder according to the limited literature target speciﬁc antigens within the nervous system . In on the topic. Unfortunately, the patient showed very mild the case of anti-Yo PCD, the most common form of PCD, improvement after receiving treatment and physical therapy. the target of the autoantibodies is Purkinje cell cytoplasmic antibody type 1 (PCA1). Anti-Yo PCD accounts for 2. Case Presentation approximately 50% of patients with PCD  and is most commonly associated with breast and gynecologic cancers. A 48-year-old female with no known past medical history Thus, females are the majority of patients with this disorder. presented with a 2-week history of an inability to ambulate, However, there have been a few cases reported with lung can- frequent falls, and slurred speech. She denied headaches, dip- cers, gastrointestinal, and prostate adenocarcinomas [3–5]. lopia, vertigo, unilateral weakness, sensory changes, bowel or Most commonly, patients present with neurological symp- bladder dysfunction, or dysphagia. The neurological exam toms, and subsequent laboratory testing and diagnostic showed dysarthria, a wide-based ataxic gait, proximal lower imaging reveals an underlying malignancy. Given the rarity extremity weakness, and hyperreﬂexia in the upper and lower of PCD, most of the knowledge of PCD is based oﬀ of case extremities bilaterally with positive cross adductors and series and reports. In this case report, we highlight an Babinski sign, but no Hoﬀman’s sign. Upper and lower limb unusual presentation of PCD with typical cerebellar signs, dysmetria was observed bilaterally. There was no weakness 2 Case Reports in Oncological Medicine tance. Other notable changes include a resolution in her Table 1: Neoplastic antibody workup. gastroparesis and improvement in her speech. Anti-Yo (titer) >1 : 640 (reference range: <1 : 40) Anti-Ri/Hu Negative (reference range: negative) 3. Discussion Low levels of antibody detected Amphiphysin (titer) (reference range: <1 : 100) Approximately 90 to 98% of patients who present with cere- bellar ataxia and found to have positive anti-Yo antibody NMDAr Negative (reference range: negative) titers have an underlying malignancy, often a gynecologic Gq1b (titer) <1 : 100 (reference range: <1 : 100) or breast cancer . Anti-Yo-associated PCD typically pre- MAG (titer) <1 : 1600 (reference range: <1 : 1600) sents with the subacute development of symptoms character- GAD65 (IU/mL) <5 (reference range: <5 IU/mL) ized by pancerebellar damage, such as severe truncal and Gliadin (units) 3 (reference range: <20 units) limb ataxia [6, 7]. Other symptoms include dysarthria, nys- HTLV1/2 Nonreactive (reference range: nonreactive) tagmus, diplopia, dysphagia, memory loss, emotional lability, and peripheral neuropathy [1, 6]. Myelopathy is primarily a clinical diagnosis diagnosed by clinical exam ﬁndings that noted in the upper extremities, and normal tone and bulk may include upper and lower motor neuron signs, such as was seen throughout all extremities. No clonus was observed. hyperactive reﬂexes and extremity weakness, and is not regu- Soft touch and pinprick sensation was intact. larly described as a ﬁnding of anti-Yo-associated PCD [1, 8]. Initial basic laboratory studies, MRI of the head and Our patient presented with a combined cerebellar and mye- spinal cord, were unrevealing, and no cerebellar atrophy or lopathic picture, which is normally seen in anti-Hu, anti-Ri, evidence of myelopathy was seen. Nerve conduction and anti-amphiphysin, and GAD65 . EMG was negative for electromyography (EMG) studies were unremarkable. Sero- peripheral neuropathy, and MRI of the brain was unreveal- logic workup revealed a positive anti-Yo titer (Table 1). ing, suggesting that her deﬁcits were due to spinal cord Other assays performed were antiganglioside and antipho- pathology . spholipid, both of which were negative. In light of the The exact mechanism of cell death in anti-Yo PCD is not serologic ﬁndings, examination of the CSF was deferred. In well understood. One proposed mechanism suggests that the order to assess for an associated malignancy, a CT abdomen coactivation of the humoral immune system and a cytotoxic and pelvis was obtained and demonstrated an abnormally T-cell response leads to selective death of Purkinje cells. enlarged left ovary and enlarged right iliac lymph nodes. Pathologically, lymphocytic perivascular cuﬃng, microglial The initial CA-125 obtained was elevated to 826.6. A pelvic activation, and CD8 lymphocyte inﬁltration of the cerebellar MRI noted a 4 cm, irregularly shaped and heterogeneously Purkinje layer are the early changes in PCD, with later enhancing left ovary, with abnormal marrow changes of changes showing loss of Purkinje cells without inﬂammation. the right sacrum and bilateral acetabula, concerning for There are currently no evidence-based guidelines avail- metastatic disease. able for treating anti-Yo-associated PCD. One study showed She underwent a bilateral salpingo-oophorectomy with antitumor therapy to be the only eﬀective method of improv- removal of a 4.5 cm pelvic mass near the left fallopian tube. ing neurological outcomes in these patients . These bene- This is typically not the standard of care for ovarian cancer, ﬁts have not been consistently observed however, and these but it was unclear at the time of surgery if the tumor was patients generally have a poor prognosis and are left bedrid- gynecologic in origin as it appeared to arise from the mesen- den with irreversible neurological symptoms. Our case dem- tery and was connected to the ﬁmbriae. Pathology revealed a onstrates that treatment has minimal beneﬁt in relieving serous carcinoma within the fallopian tube and mass. A neurological symptoms, but some deﬁcits may be recoverable. repeat preoperative CA-125 was elevated to 2,198.4 and decreased to 281.9 post-operation. CT and SPECT after sur- gery were negative for metastatic disease. She was also found Conflicts of Interest to have a germline BRCA mutation at this time. A diagnostic The authors declare that they have no conﬂict of interest. mammogram demonstrated benign ﬁndings. She was diagnosed with primary peritoneal carcinoma References complicated by anti-Yo-related PCD. The Gynecology- Oncology service believed that she likely would not tolerate  A. Venkatraman and P. Opal, “Paraneoplastic cerebellar a complete staging surgery due to her poor performance degeneration with anti-Yo antibodies – a review,” Annals of status and medical comorbidities; thus, the decision was Clinical Translational Neurology, vol. 3, no. 8, pp. 655–663, made to proceed with adjuvant chemotherapy since there was no known residual disease. She received one cycle of  T. J. O'Brien, B. Pasaliaris, A. D'Apice, and E. Byrne, “Anti-Yo carboplatin alone due to her poor performance status and positive paraneoplastic cerebellar degeneration: a report of postoperative gastroparesis necessitating a gastrojejunost- three cases and review of the literature,” Journal of Clinical omy tube, followed by 5 cycles of carboplatin and paclitaxel Neuroscience, vol. 2, no. 4, pp. 316–320, 1995. for 6 total cycles of adjuvant chemotherapy with remission  L. Hasadsri, J. Lee, B. H. Wang, L. Yekkirala, and M. Wang, conﬁrmed by a nadir in her CA-125. After one and a half “Anti-yo associated paraneoplastic cerebellar degeneration in years of follow-up, she remains without evidence of disease a man with large cell cancer of the lung,” Case Reports in Neu- and is able to move her extremities and transfer with assis- rological Medicine, vol. 2013, Article ID 725936, 5 pages, 2013. Case Reports in Oncological Medicine 3  B. Meglic, F. Graus, and A. Grad, “Anti-Yo-associated paraneoplastic cerebellar degeneration in a man with gastric adenocarcinoma,” Journal of the Neurological Sciences, vol. 185, no. 2, pp. 135–138, 2001.  I. J. Sutton, C. J. Fursdon Davis, M. M. Esiri, S. Hughes, E. R. Amyes, and A. Vincent, “Anti-Yo antibodies and cerebellar degeneration in a man with adenocarcinoma of the esopha- gus,” Annals of Neurology, vol. 49, no. 2, pp. 253–257, 2001.  K. P. MD, M. K. Rosenblum, H. K. MS, and J. B. Posner, “Paraneoplastic cerebellar degeneration. I. A clinical analysis of 55 anti-Yo antibody-positive patients,” Neurology, vol. 42, no. 10, pp. 1931–1937, 1992.  I. Rojas, F. Graus, F. Keime-Guibert et al., “Long-term clinical outcome of paraneoplastic cerebellar degeneration and anti- Yo antibodies,” Neurology, vol. 55, no. 5, pp. 713–715, 2000.  W. O. Tobin and S. J. Pittock, “Autoimmune neurology of the central nervous system,” CONTINUUM: Lifelong Learning in Neurology, vol. 23, no. 3, pp. 627–653, 2017.  W. B. Young, “The clinical diagnosis of myelopathy,” Seminars in Ultrasound, CT, and MR, vol. 15, no. 3, pp. 250–254, 1994.  P. M. Candler, P. E. Hart, M. Barnett, R. Weil, and J. H. Rees, “A follow up study of patients with paraneoplastic neurologi- cal disease in the United Kingdom,” Journal of Neurology, Neurosurgery, and Psychiatry, vol. 75, no. 10, pp. 1411–1415, 2004. MEDIATORS of INFLAMMATION The Scientific Gastroenterology Journal of World Journal Research and Practice Diabetes Research Disease Markers Hindawi Hindawi Publishing Corporation Hindawi Hindawi Hindawi Hindawi www.hindawi.com Volume 2018 http://www www.hindawi.com .hindawi.com V Volume 2018 olume 2013 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 International Journal of Journal of Immunology Research Endocrinology Hindawi Hindawi www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 Submit your manuscripts at www.hindawi.com BioMed PPAR Research Research International Hindawi Hindawi www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 Journal of Obesity Evidence-Based Journal of Journal of Stem Cells Complementary and Ophthalmology International Alternative Medicine Oncology Hindawi Hindawi Hindawi Hindawi Hindawi www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2013 Parkinson’s Disease Computational and Behavioural Mathematical Methods AIDS Oxidative Medicine and in Medicine Neurology Research and Treatment Cellular Longevity Hindawi Hindawi Hindawi Hindawi Hindawi www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018
Case Reports in Oncological Medicine – Hindawi Publishing Corporation
Published: Jan 9, 2020
Access the full text.
Sign up today, get DeepDyve free for 14 days.