scholarly journals Pathogenesis of anti-Yo positive paraneoplastic cerebellar degeneration

SURG Journal ◽  
1969 ◽  
Vol 1 (1) ◽  
pp. 42-50
Author(s):  
Laura Pepler ◽  
Carol Armstrong
Keyword(s):  
Breast Cancer ◽  
Immune Response ◽  
Animal Model ◽  
Cytotoxic T Lymphocytes ◽  
Symptomatic Treatment ◽  
Cerebellar Degeneration ◽  
Paraneoplastic Cerebellar Degeneration ◽  
Immune Mediated ◽  
Suitable Animal Model ◽  
Pathogenic Process

Paraneoplastic cerebellar degeneration (PCD) is a neurological disorder affecting the cerebellum and is believed to be immune-mediated. Anti-Yo PCD is associated with gynaecological and breast cancer tumours and the presence of these tumours is believed to invoke an immune response. The immune response includes both anti-Yo antibodies and cytotoxic T-lymphocytes that are specific for the Cdr2 antigen expressed in Purkinje cells of the cerebellum. Although both have been implicated in the pathogenic manifestation of PCD neither have been shown to be directly involved. The detection of the anti-Yo antibody greatly aids in the diagnosis of PCD and helps in uncovering the underlying neoplasm before it becomes symptomatic. Treatment of PCD has been targeted at suppressing the immune response but has been unsuccessful so far. The lack of a suitable animal model makes it difficult to study the pathogenic process directly leaving many questions unanswered about PCD pathogenesis.

Paraneoplastic cerebellar degeneration diagnosed by anti-Yo determination in a young woman with early breast cancer

2020 ◽  
Vol 13 (8) ◽  
pp. e233863 ◽  
Author(s):  
Oriol Mirallas ◽  
María Alejandra Rezqallah Arón ◽  
Nadia Saoudi Gonzalez ◽  
Santiago Escrivá-de-Romaní
Keyword(s):  
Breast Cancer ◽  
Early Breast Cancer ◽  
Brain Mri ◽  
Neoadjuvant Treatment ◽  
Cerebellar Degeneration ◽  
Paraneoplastic Cerebellar Degeneration ◽  
Her2 Positive ◽  
Limb Weakness ◽  
Fluid Analysis ◽  
Positron Emission

A 44-year-old woman diagnosed with a HER2 positive early breast cancer, receiving neoadjuvant treatment with paclitaxel and targeted agents, trastuzumab together with pertuzumab, presented to the emergency room with gait instability and upper right limb weakness. The neurological examination was compatible with cerebellar alteration showing right dissymmetry of the finger-nose and heel-knee manoeuvre. A head CT and a brain MRI were performed and negative. The electromyography showed alterations of the pyramidal pathway and somatosensory pathway. In order to determine the cause of the cerebellar affection, a lumbar puncture was performed. The cerebrospinal fluid analysis was non-specific, but the antineuronal anti-Yo antibody was positive, being diagnosed of a paraneoplastic cerebellar degeneration (PCD). A positron emission tomography CT ruled out metastatic disease. The patient completed four cycles of antiHER2 blockade and weekly paclitaxel, achieving a complete pathological response. One year later, she maintains a complete remission but the PCD still prevails.

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

2019 ◽  
Vol 12 (8) ◽  
pp. e229777 ◽  
Author(s):  
Eman Butt ◽  
John A Tadross ◽  
Karan R Chadda ◽  
John Latimer
Keyword(s):  
Early Recognition ◽  
Cerebellar Degeneration ◽  
Paraneoplastic Cerebellar Degeneration ◽  
Serous Carcinoma ◽  
High Grade ◽  
Neurological Disability ◽  
Immune Mediated ◽  
Underlying Malignancy ◽  
After Cancer ◽  
Vascular Accidents

This case describes a 69-year-old woman, who presented with rapidly progressive cerebellar symptoms and unintentional weight loss. Full neurological assessment excluded space-occupying lesions, vascular accidents and infection. Surprisingly, a chest, abdomen and pelvis CT showed a left hemipelvis mass, which was subsequently biopsied. A high-grade serous carcinoma of tubo-ovarian origin was found, diagnosing paraneoplastic cerebellar degeneration (PCD) secondary to this. The exact mechanism is not known, but is thought to be immune-mediated. In cases of PCD, after cancer treatment, the neurological disability stabilises to a severe level and will unfortunately be lifelong. Our patient continues to make great progress with intensive rehabilitation for her ongoing balance issues. Early recognition of PCD can lead to a prompt diagnosis of the underlying malignancy and hence subsequent management. This can at least limit the extent of the neurological disability of the disease and increase the survival rate from cancer.

scholarly journals Paraneoplastic Neurological Syndromes: Study of Prevalence in a Province of the Lombardy Region, Italy

2020 ◽  
Vol 9 (10) ◽  
pp. 3105
Author(s):  
Lorenzo Lorusso ◽  
Vincenza Precone ◽  
Daniela Ferrari ◽  
Gaelle K. Ngonga ◽  
Antonio Giampiero Russo ◽  
...  
Keyword(s):  
Hospital Admissions ◽  
Cerebellar Degeneration ◽  
Paraneoplastic Cerebellar Degeneration ◽  
Peripheral Nervous System Diseases ◽  
Paraneoplastic Neurological Syndromes ◽  
Lombardy Region ◽  
Immune Mediated ◽  
Classification Of Diseases ◽  
International Statistical Classification

Paraneoplastic neurological syndromes (PNSs) are a heterogeneous group of rare immune-mediated diseases associated with cancer. The aim of this study was to investigate the prevalence of PNSs in the province of Brescia. PNS prevalence was calculated using the Lombardy regional hospital admission records from 1998 to 2003. We used the website “Epidemiologic and Economic Atlas of Hospital Activities in Lombardy” and the “International Statistical Classification of Diseases and Related Health Problems”. In the province of Brescia, we found 54 cases of PNSs, 29 with subacute neuropathies, five with paraneoplastic cerebellar degeneration and 20 with encephalomyelitis. Peripheral nervous system diseases were the most frequent neurological disorders. In Lombardy, the number of PNS patients admitted was 322 (133 with encephalomyelitis, 21 with paraneoplastic cerebellar degeneration, 166 with polyneuropathies and two with optic degeneration). In Lombardy, the prevalence of PNSs was 25 in 100,000 hospital admissions and 5.92 in 100,000 for the Lombardy population. Our results show a discrete presence of PNS patients in the province of Brescia and in the Lombardy region as a whole.

scholarly journals Anti-Yo Mediated Paraneoplastic Cerebellar Degeneration Associated with Pseudobulbar Affect in a Patient with Breast Cancer

2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Allison N. Martin ◽  
Patrick M. Dillon ◽  
David E. Jones ◽  
David R. Brenin ◽  
David A. Lapides
Keyword(s):  
Breast Cancer ◽  
Paraneoplastic Syndrome ◽  
Radical Mastectomy ◽  
Cerebellar Degeneration ◽  
Paraneoplastic Syndromes ◽  
Paraneoplastic Cerebellar Degeneration ◽  
Modified Radical Mastectomy ◽  
Stage Iiia ◽  
Pseudobulbar Affect ◽  
New Onset

Paraneoplastic cerebellar degeneration (PCD) is a rare anti-Yo mediated paraneoplastic syndromes rarely that is infrequently associated with breast cancer. We present a case of a 52-year-old female presenting with diplopia, gait instability, dysarthria, dysphagia, nystagmus, and, most notably, new onset paroxysmal episodes of uncontrollable crying concerning for pseudobulbar affect (PBA). Serologic testing showed anti-Yo antibodies. The patient was found to have stage IIIA breast cancer as the inciting cause of the paraneoplastic syndrome. The patient was treated with neoadjuvant chemotherapy, modified radical mastectomy, adjuvant Herceptin, and pertuzumab. She was given IVIG for paraneoplastic syndrome, antidepressants, and dextromethorphan-quinidine (Nuedexta), the first FDA-approved therapy for PBA. With multimodality therapy, she demonstrated significant improvement in neurologic and mood symptoms associated with PCD and PBA.

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