Paraneoplastic Cerebellar Degeneration with Anti-CV2/CRMP5 Antibodies in Ovarian Cancer: Case Report and Review of the Literature

Abstract

Paraneoplastic neurological syndromes (PNS) are rare presentations of an underlying oncological disease and more unusual during an oncological disease. They most likely present in small-cell lung carcinomas and thymomas, but present in <1% of the gynecological neoplasms. Acknowledging the pathophysiology is essential for management, explaining its clinical presentation, and future research. We present a patient with an underlying gynecological cancer that during her disease developed a PNS with an unusual autoantibody (anti-CV2/CRMP5) mediating the disease. We report a case of a 62-year-old female diagnosed with ovarian cancer who in the course of her disease developed neurological symptoms associated with cerebellar degeneration. After ruling out differential diagnoses such as metastases, a PNS was suspected and studied, in which anti-CV2/CRMP5 antibodies were positive. With her clinical presentation, radiological features, autoantibody positivity on cerebrospinal fluid, and an underlying oncological disease, cerebellar degeneration was diagnosed. The pathophysiology of PNS is not fully understood; therefore, its diagnosis and management are complex. Diagnosis is based on clinical presentation and specific antibodies associated. Unfortunately, patients have a bad prognosis and diminished quality of life, and therefore a multidisciplinary approach is needed. It is important to mention that the presentation of PNS does not mandatorily appear before the diagnosis of cancer, and multiple cases have been reported in which patients with an underlying oncological disease develop these syndromes. As medical oncologists and neurologists, we must consider and study these syndromes as a possible etiology in cases with an underlying cancer who develop neurological symptoms in the course of their disease.

Gynecologic clear cell carcinoma and paraneoplastic cerebellar Degeneration: A literature review and case study

“…Table 1 highlights the previously reported primary gynecologic malignancies associated with PCD, along with patient symptoms, imaging findings, and outcomes ( Cao et al, 1999 , Liontos et al, 2021 , Marchand et al, 2007 , Campero and Selman, 2017 , Tanaka et al, 2005 , Johns et al, 1999 , Erez et al, 2007 , Juárez-Vignon Whaley et al, 2021 , Elomrani et al, 2014 , Negishi et al, 2014 , Panegyres and Graves, 2012 , Russo et al, 2013 , Butt et al, 2019 ). Here, we present a patient diagnosed with PCD in the setting of gynecologic clear cell carcinoma and highlight how symptomatology and neurologic outcomes in this disease process may be associated and provide clinicians with management considerations.…”

Paraneoplastic neurological syndrome caused by cystitis glandularis: A case report and literature review

“…Paraneoplastic neurological syndrome (PNS) comprises a group of clinical syndromes that occur in the presence of systemic malignant tumors[ 1 - 3 ]. PNS can affect any part of the central and peripheral nervous system[ 4 - 6 ]. Most paraneoplastic syndromes are caused by autoimmune cross-reactions between tumor antigens and proteins expressed in the nervous system[ 7 - 11 ].…”