Standard Article

Paraneoplastic Neurologic Syndromes

  1. Peter Maat,
  2. Peter AE Sillevis Smitt

Published Online: 15 SEP 2009

DOI: 10.1002/9780470015902.a0021410

eLS

eLS

How to Cite

Maat, P. and Sillevis Smitt, P. A. 2009. Paraneoplastic Neurologic Syndromes. eLS. .

Author Information

  1. Erasmus University Medical Center, Erasmus MC‘s-Gravendijkwal 230, Rotterdam, The Netherlands

Publication History

  1. Published Online: 15 SEP 2009

Abstract

Paraneoplastic neurologic syndromes (PNS) are remote effects of cancer. Immunological factors appear important in the pathogenesis of PNS since the detection of antineuronal autoantibodies. Detection of paraneoplastic antibodies is very helpful in diagnosing an unexplained neurological syndrome as paraneoplastic and in directing the search towards the underlying neoplasm. There are seven well-characterized paraneoplastic antibodies reactive with the onconeural Hu, Yo, Ri, Ma2, CV2, amphiphysin and recoverin antigens. Recently, anti-VGKC and anti-NMDA receptor antibodies were identified in patients with limbic encephalitis (LE). Thirty per cent of patients with anti-VGKC and LE have an underlying tumour, while most patients with anti-NMDAR and LE are young women with an ovarian teratoma. Both VGKC- and NMDAR-related LE respond well to immunotherapy. In contrast, the effect of immunotherapy in patients with PNS associated with antibodies against the intracellular onconeural antigens is disappointing. These patients benefit most from early detection and treatment of the underlying tumour.

Keywords:

  • paraneoplastic syndromes;
  • tumour autoimmunity;
  • onconeural antibodies