Inflammatory infiltrates and complete absence of Purkinje cells in anti-Yo-associated paraneoplastic cerebellar degeneration Journal Article


Authors: Verschnuren, J.; Chuang, L.; Rosenblum, M. K.; Lieberman, F.; Pryor, A.; Posner, J. B.; Dalmau, J.
Article Title: Inflammatory infiltrates and complete absence of Purkinje cells in anti-Yo-associated paraneoplastic cerebellar degeneration
Abstract: We studied the nervous systems and tumors of two patients with anti-Yo-associated paraneoplastic cerebellar degeneration (PCD). In both patients the underlying tumor was an ovarian adenocarcinoma that expressed Yo antigens and contained extensive infiltrates of lymphocytes and plasma cells. The major central nervous system findings were a complete loss of cerebellar Purkinje cells with Bergmann astrogliosis. One patient had inflammatory infiltrates in the medulla and pens, and moderate axonal loss and demyelination involving the spinal cord. No inflammatory infiltrates were identified in the cerebrum, cerebellum or brain-stem of the other patient. Using quantitative Western blot analysis, deposits of anti-lie IgG could not be demonstrated in the nervous system, possibly as a result of the loss of cells expressing Yo antigens. The detection of the anti-Yo antibody as a common marker of PCD in one patient with inflammatory infiltrates and another without infiltrates suggests that some PCD pathologically classified as 'non-inflammatory' may represent a final bum-out stage of a cellular immune-mediated disorder. Our findings indicate that Purkinje cells are the main, but not necessarily the exclusive, targets of this disorder.
Keywords: adult; human tissue; middle aged; dna-binding proteins; case report; ovarian cancer; ovary adenocarcinoma; ovarian neoplasms; purkinje cell; purkinje cells; neoplasm proteins; nerve tissue proteins; plasma cell; spinal cord; cell movement; autoantigens; inflammatory infiltrate; lymphocytic infiltration; fatal outcome; adenocarcinoma, papillary; nerve degeneration; demyelination; pons; cerebellar diseases; paraneoplastic cerebellar degeneration; paraneoplastic syndromes; medulla oblongata; astrocytosis; cerebellum degeneration; humans; human; female; priority journal; article; anti-yo antibodies
Journal Title: Acta Neuropathologica
Volume: 91
Issue: 5
ISSN: 0001-6322
Publisher: Springer  
Date Published: 1996-04-01
Start Page: 519
End Page: 525
Language: English
DOI: 10.1007/s004010050460
PUBMED: 8740233
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 22 November 2017 -- Source: Scopus
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  1. Marc Rosenblum
    424 Rosenblum
  2. Jerome B Posner
    211 Posner
  3. Josep O Dalmau
    101 Dalmau