Paraneoplastic Cerebellar Degeneration (PCD) Associated with PCA-1 Antibodies in Established Cancer Patients

Abstract Paraneoplastic cerebellar degeneration (PCD) is a rare set of neurological disorders arising from tumor-associated autoimmunity against antigens within the cerebellum. Anti-Purkinje cell cytoplasmic antibody 1 (PCA-1), or anti-Yo, is the most commonly linked antibody and is classically associated with breast and ovarian cancers. Here we report case series of PCA-1 associated PCD in patients with known breast or ovarian cancer diagnosis not receiving immunotherapy. These cases highlight recognizing PCA-1 paraneoplastic syndrome triggered by cytotoxic chemotherapy, surgery, tumor recurrence and associated with development of second cancer. Diagnosis of the syndrome requires neurological workup with lumbar puncture (LP) with cerebrospinal fluids (CSF) studies, serum and CSF paraneoplastic antibody panel, and neuroimaging. Inpatient admission for prompt workup and initiation of treatment is recommended. Treatment most commonly includes immunosuppression with corticosteroids, plasmapheresis, and/or intravenous immune globulin (IVIG); however, we postulate that other immune modulating treatments may warrant consideration. In established cancer patients developing this syndrome, workup and treatment of tumor recurrence or development of second malignancy is recommended. These cases highlight the need for early recognition of the syndrome in patients receiving non immune based chemotherapy, for prompt workup and treatment.

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Combined Cerebellar and Spinal Cord Deficits Caused by an Underlying Gynecologic Malignancy

Case Reports in Oncological Medicine ◽

10.1155/2020/9021843 ◽

2020 ◽

Vol 2020 ◽

pp. 1-3

Author(s):

Tamer Othman ◽

Moshe-Samuel Hendizadeh ◽

Ritika Vankina ◽

Susan Park ◽

Phyllis Kim

Keyword(s):

Cancer Diagnosis ◽

Gynecologic Cancer ◽

Case Series ◽

Autoimmune Disorder ◽

Cerebellar Degeneration ◽

Newly Diagnosed ◽

Gynecologic Malignancy ◽

Underlying Malignancy ◽

Gynecologic Tumors ◽

Unusual Finding

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.

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

BMJ Case Reports ◽

10.1136/bcr-2019-229777 ◽

2019 ◽

Vol 12 (8) ◽

pp. e229777 ◽

Cited By ~ 2

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.

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Paraneoplastic Cerebellar Degeneration (PCD) associated with PCA-1 antibodies in established cancer patients

Journal of Neuro-Oncology ◽

10.1007/s11060-021-03779-7 ◽

2021 ◽

Author(s):

Michael J. Lehner ◽

Jinesh S. Gheeya ◽

Bilal A. Siddiqui ◽

Sudhakar Tummala

Keyword(s):

Cancer Patients ◽

Cerebellar Degeneration ◽

Paraneoplastic Cerebellar Degeneration

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Anti-Yo antibody–mediated paraneoplastic cerebellar degeneration associated with cognitive affective syndrome in a patient with breast cancer: a case report and literature review

Current Oncology ◽

10.3747/co.25.4106 ◽

2018 ◽

Vol 25 (6) ◽

Cited By ~ 6

Author(s):

M. Le May ◽

S. Dent

Keyword(s):

Breast Cancer ◽

Targeted Therapy ◽

Early Recognition ◽

Growth Factor Receptor ◽

Cerebellar Degeneration ◽

Paraneoplastic Cerebellar Degeneration ◽

Her2 Positive ◽

Epidermal Growth ◽

Estrogen And Progesterone Receptor ◽

Positive Breast Cancer

Breast cancer is the most common cancer in women, with 15%–25% of those tumours overexpressing the human epidermal growth factor receptor 2 (her2), which is associated with more aggressive disease. On rare occasions, patients present with a paraneoplastic syndrome months to years before their cancer diagnosis. Paraneoplastic cerebellar degeneration (pcd) is associated with fewer than 1% of cancers and is strongly associated with breast and gynecologic malignancies. Anti-Yo antibody is the antibody most frequently identified with the syndrome, and it is associated with a very poor prognosis. Recent studies have implicated a relationship between overexpression of her2 and anti-Yo–mediated pcd. Current pcd treatments include tumour removal, chemotherapy, targeted therapy, and immune-suppressive treatments. Outcomes of pcd are typically poor, and no guidelines for treatment currently exist. Early recognition followed by rapid initiation of treatment remains the cornerstone of therapy.Here, we present a case of anti-Yo-antibody pcd secondary to estrogen and progesterone receptor–negative, her2-positive breast cancer. Despite treatment with mastectomy, chemotherapy, and her2-targeted therapy, no significant neurologic improvement was achieved, and cerebellar cognitive affective syndrome subsequently developed.

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Anti-Yo-Associated Paraneoplastic Cerebellar Degeneration: Case Series and Review of Literature

Cureus ◽

10.7759/cureus.20203 ◽

2021 ◽

Author(s):

Mathew Chatham ◽

Polly Niravath

Keyword(s):

Case Series ◽

Cerebellar Degeneration ◽

Paraneoplastic Cerebellar Degeneration ◽

Review Of Literature

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Breast cancer associated with paraneoplastic cerebellar degeneration: A case series

Indian Journal of Cancer ◽

10.4103/ijc.ijc_132_19 ◽

2020 ◽

Vol 0 (0) ◽

pp. 0

Author(s):

Ajay Gogia ◽

Ilavarasi Vanidassane ◽

Vinod Raina ◽

Priya Tiwari

Keyword(s):

Breast Cancer ◽

Case Series ◽

Cerebellar Degeneration ◽

Paraneoplastic Cerebellar Degeneration

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Thrombosis in Cancer Patients Treated with Hematopoietic Growth Factors - A Meta-Analysis

Thrombosis and Haemostasis ◽

10.1055/s-0038-1650276 ◽

1996 ◽

Vol 75 (02) ◽

pp. 368-371 ◽

Cited By ~ 51

Author(s):

T Barbul ◽

G Finazzi ◽

A Grassi ◽

R Marchioli

Keyword(s):

Cancer Patients ◽

Case Reports ◽

Meta Analysis ◽

Case Series ◽

Hematopoietic Growth Factors ◽

Patients With Cancer ◽

Cytotoxic Treatment ◽

Pertinent Data ◽

Thrombotic Complications ◽

Vascular Occlusions

SummaryHematopoietic colony-stimulating factors (CSFs) are largely used in patients with cancer undergoing cytotoxic treatment to accelerate neutrophil recovery and decrease the incidence of febrile neutropenia. Clinical practice guidelines for their use have been recently established (1), taking into account clinical benefit, but also cost and toxicity. Vascular occlusions have been recently reported among the severe reactions associated with the use of CSFs, in anedoctal case reports (2, 3), consecutive case series (4) and randomized clinical trial (5, 6). However, the role of CSFs in the pathogenesis of thrombotic complications is difficult to ascertain, because pertinent data are scanty and widely distributed over a number of heterogenous investigations. We report here a systematic review of relevant articles, with the aims to estimate the prevalence of thrombosis associated with the use of CSFs and to assess if this rate is significantly higher than that observed in cancer patients not receiving CSFs.

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