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

  • Clinical Study
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Abstract

Introduction

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.

Methods

Medical records of patients at our institution who developed PCA-1 associated PCD were reviewed. Clinical information, including cancer history, cancer-directed treatment, and serum and CSF titers of PCA-1 antibody were extracted.

Cases

We report a series of cases of PCA-1 associated PCD in patients with known breast or ovarian cancer diagnosis not receiving immunotherapy. These cases highlight aspects of PCA-1 paraneoplastic syndrome such as triggering by cytotoxic chemotherapy or surgery, the possibility of tumor recurrence and the association with development of a second cancer.

Discussion

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.

Conclusion

These cases highlight the need for early recognition of the syndrome in patients receiving nonimmune based chemotherapy, for prompt workup and treatment.

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Funding

The authors did not receive support from any organization for the submitted work.

Author information

Authors and Affiliations

  1. Department of Internal Medicine, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, USA

    Michael J. Lehner & Jinesh S. Gheeya

  2. Department of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Bilal A. Siddiqui

  3. Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd., Unit 0431, Houston, TX, 77030, USA

    Sudhakar Tummala

Authors
  1. Michael J. Lehner
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  2. Jinesh S. Gheeya
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  3. Bilal A. Siddiqui
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  4. Sudhakar Tummala
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Contributions

MJL and JSG drafted the initial manuscript. BAS and ST provided critical review of the manuscript. ST provided clinical data and supervised the effort. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Sudhakar Tummala.

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Conflict of interest

MJL, JSG, BAS and ST report no financial disclosures.

Ethical approval

The committee reviewed this submission and assigned a determination of Not Human Research. Internal code is 2021-0192.

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The committee reviewed this submission and assigned a determination of Not Human Research.

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The committee reviewed this submission and assigned a determination of Not Human Research.

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Lehner, M.J., Gheeya, J.S., Siddiqui, B.A. et al. Paraneoplastic Cerebellar Degeneration (PCD) associated with PCA-1 antibodies in established cancer patients. J Neurooncol 153, 441–446 (2021). https://doi.org/10.1007/s11060-021-03779-7

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  • DOI: https://doi.org/10.1007/s11060-021-03779-7

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