Autoimmune response of patients with paraneoplastic cerebellar degeneration to a Purkinje cell cytoplasmic protein antigen

Kurt Jaeckle, Francesc Graus, Alan Houghton, Carlos Cardon‐Cardo, Surl L. Nielsen, Jerome B. Posner

Research output: Contribution to journalArticle

160 Citations (Scopus)

Abstract

Sera from 6 of 12 patients with paraneoplastic cerebellar degeneration (PCD) contained anti–Purkinje cell antibodies, as determined by indirect immunofluorescence on frozen sections of normal human cerebellum. Samples of cerebrospinal fluid from 2 of the patients with serum antibodies were tested, and both specimens contained anti–Purkinje cell antibody. The anti‐Purkinje cell antibodies were polyclonal, fixed complement, and were present in all patients at serum dilutions of 1:1,000 or greater. Antibody activity could not be suppressed by preabsorption of sera with human or animal brain and tissue powders or with fresh crude human cerebellar extracts. No anti–Purkinje cell antibodies were detected in control sera from 167 neurologically normal cancer patients, 32 normal volunteers, 10 patients with other causes of cerebellar degeneration, or 8 patients with other paraneoplastic neurological diseases. Preliminary evidence suggests that the Purkinje antigen is a protein that is often concentrated in the periphery of the cytoplasm in disc‐shaped structures. Patients with antibodies often developed signs of PCD near the time of detection of the tumor and had relentless progression of neurological disease. Patients without antibodies frequently had cancer for months to years before PCD developed, and often had spontaneous stabilization of neurological disease with time. Four patients without and 3 patients with antibodies underwent plasmapheresis without response.

Original languageEnglish (US)
Pages (from-to)592-600
Number of pages9
JournalAnnals of Neurology
Volume18
Issue number5
DOIs
StatePublished - Jan 1 1985

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Paraneoplastic Cerebellar Degeneration
Purkinje Cells
Autoimmunity
Antigens
Antibodies
Proteins
Serum
Neoplasms
Plasmapheresis
Frozen Sections
Indirect Fluorescent Antibody Technique
Powders
Cerebellum
Cerebrospinal Fluid
Disease Progression
Healthy Volunteers
Cytoplasm

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology

Cite this

Jaeckle, Kurt ; Graus, Francesc ; Houghton, Alan ; Cardon‐Cardo, Carlos ; Nielsen, Surl L. ; Posner, Jerome B. / Autoimmune response of patients with paraneoplastic cerebellar degeneration to a Purkinje cell cytoplasmic protein antigen. In: Annals of Neurology. 1985 ; Vol. 18, No. 5. pp. 592-600.
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Jaeckle, K, Graus, F, Houghton, A, Cardon‐Cardo, C, Nielsen, SL & Posner, JB 1985, 'Autoimmune response of patients with paraneoplastic cerebellar degeneration to a Purkinje cell cytoplasmic protein antigen', Annals of Neurology, vol. 18, no. 5, pp. 592-600. https://doi.org/10.1002/ana.410180513

Autoimmune response of patients with paraneoplastic cerebellar degeneration to a Purkinje cell cytoplasmic protein antigen. / Jaeckle, Kurt; Graus, Francesc; Houghton, Alan; Cardon‐Cardo, Carlos; Nielsen, Surl L.; Posner, Jerome B.

In: Annals of Neurology, Vol. 18, No. 5, 01.01.1985, p. 592-600.

Research output: Contribution to journalArticle

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