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Paraneoplastic cerebellar degeneration with anti‐Yo antibodies – a review
The ataxic syndrome associated with Anti‐Yo antibody, or Purkinje cell cytoplasmic antibody type 1 (PCA1), is the most common variant of paraneoplastic cerebellar degeneration (PCD). The typical presentation involves the subacute development of pancerebellar deficits with a clinical plateau within 6...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley and Sons Inc.
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4999597/ https://www.ncbi.nlm.nih.gov/pubmed/27606347 http://dx.doi.org/10.1002/acn3.328 |
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author | Venkatraman, Anand Opal, Puneet |
author_facet | Venkatraman, Anand Opal, Puneet |
author_sort | Venkatraman, Anand |
collection | PubMed |
description | The ataxic syndrome associated with Anti‐Yo antibody, or Purkinje cell cytoplasmic antibody type 1 (PCA1), is the most common variant of paraneoplastic cerebellar degeneration (PCD). The typical presentation involves the subacute development of pancerebellar deficits with a clinical plateau within 6 months. The vast majority of cases have been reported in women with pelvic or breast tumors. Magnetic resonance imaging of the brain is often normal in the early stages, with cerebellar atrophy seen later. The underlying mechanism is believed to be an immunological reaction to cerebellar degeneration‐related protein 2 (CDR2), a protein usually found in the cerebellum that is ectopically produced by tumor cells. Although both B‐ and T‐cell abnormalities are seen, there is debate about the relative importance of the autoantibodies and cytotoxic T lymphocytes in the neuronal loss. Cerebrospinal fluid abnormalities, primarily elevated protein, lymphocytic pleocytosis, and oligoclonal bands, are common in the early stages. The low prevalence of this condition has not allowed for large‐scale randomized controlled trials. Immunotherapies, such as steroids, intravenous immune globulins, and plasma exchange, have been extensively used in managing this condition, with limited success. Although some reports indicate benefit from antitumor therapies like surgery and chemotherapy, this has not been consistently observed. The prognosis for anti‐Yo PCD is almost uniformly poor, with most patients left bedridden. Further studies are required to clarify the pathophysiology and provide evidence‐based treatment options. |
format | Online Article Text |
id | pubmed-4999597 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | John Wiley and Sons Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-49995972016-09-07 Paraneoplastic cerebellar degeneration with anti‐Yo antibodies – a review Venkatraman, Anand Opal, Puneet Ann Clin Transl Neurol Review Article The ataxic syndrome associated with Anti‐Yo antibody, or Purkinje cell cytoplasmic antibody type 1 (PCA1), is the most common variant of paraneoplastic cerebellar degeneration (PCD). The typical presentation involves the subacute development of pancerebellar deficits with a clinical plateau within 6 months. The vast majority of cases have been reported in women with pelvic or breast tumors. Magnetic resonance imaging of the brain is often normal in the early stages, with cerebellar atrophy seen later. The underlying mechanism is believed to be an immunological reaction to cerebellar degeneration‐related protein 2 (CDR2), a protein usually found in the cerebellum that is ectopically produced by tumor cells. Although both B‐ and T‐cell abnormalities are seen, there is debate about the relative importance of the autoantibodies and cytotoxic T lymphocytes in the neuronal loss. Cerebrospinal fluid abnormalities, primarily elevated protein, lymphocytic pleocytosis, and oligoclonal bands, are common in the early stages. The low prevalence of this condition has not allowed for large‐scale randomized controlled trials. Immunotherapies, such as steroids, intravenous immune globulins, and plasma exchange, have been extensively used in managing this condition, with limited success. Although some reports indicate benefit from antitumor therapies like surgery and chemotherapy, this has not been consistently observed. The prognosis for anti‐Yo PCD is almost uniformly poor, with most patients left bedridden. Further studies are required to clarify the pathophysiology and provide evidence‐based treatment options. John Wiley and Sons Inc. 2016-06-30 /pmc/articles/PMC4999597/ /pubmed/27606347 http://dx.doi.org/10.1002/acn3.328 Text en © 2016 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association. This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial‐NoDerivs (http://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made. |
spellingShingle | Review Article Venkatraman, Anand Opal, Puneet Paraneoplastic cerebellar degeneration with anti‐Yo antibodies – a review |
title | Paraneoplastic cerebellar degeneration with anti‐Yo antibodies – a review |
title_full | Paraneoplastic cerebellar degeneration with anti‐Yo antibodies – a review |
title_fullStr | Paraneoplastic cerebellar degeneration with anti‐Yo antibodies – a review |
title_full_unstemmed | Paraneoplastic cerebellar degeneration with anti‐Yo antibodies – a review |
title_short | Paraneoplastic cerebellar degeneration with anti‐Yo antibodies – a review |
title_sort | paraneoplastic cerebellar degeneration with anti‐yo antibodies – a review |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4999597/ https://www.ncbi.nlm.nih.gov/pubmed/27606347 http://dx.doi.org/10.1002/acn3.328 |
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