This Article Full Text Full Text (PDF) CME: Take the course for this article: Managing Paraneoplastic Neurological Disorders eLetters: Submit a response to this article Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services E-mail this article link to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Reprints/Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by de Beukelaar, J. W. Articles by Smitt, P. A. S. Search for Related Content PubMed PubMed Citation Articles by de Beukelaar, J. W. Articles by Smitt, P. A. S.

Managing Paraneoplastic Neurological Disorders

Erasmus University Medical Center, Rotterdam, The Netherlands

Key Words. Paraneoplastic neurological syndromes • Cancer • Autoimmunity • Autoantibodies • Onconeural antigens

Correspondence: Peter Sillevis Smitt, M.D., Ph.D., Department of Neurology, Room H664, Erasmus University Medical Center, Dr Molewater 40, 3015 GD, Rotterdam, The Netherlands. Telephone: 00-31-10-4633327; Fax: 00-31-10-4632156; e-mail: p.sillevissmitt{at}erasmusmc.nl

Paraneoplastic neurological syndromes (PNS) are remote effects of cancer that are not caused by invasion of the tumor or its metastases. Immunologic factors appear important in the pathogenesis of PNS because antineuronal autoantibodies and T-cell responses against nervous system antigens have been defined for many of these disorders. The immunologic response is elicited by the ectopic expression of neuronal antigens by the tumor. Expression of these so-called "onconeural" antigens is limited to the tumor and the nervous system and sometimes also the testis. At the time of presentation of the neurological symptoms, most patients have not yet been diagnosed with cancer. Detection of paraneoplastic antibodies is extremely helpful in diagnosing an otherwise unexplained and often rapidly progressive neurological syndrome as paraneoplastic. In addition, the paraneoplastic antibodies may also direct the search for an underlying neoplasm. On the other hand, in patients known to have cancer, the presentation of a PNS may herald recurrence of the tumor or a second tumor. The number of paraneoplastic antibodies is still growing, and at least seven of these can now be considered well characterized. Based on the clinical syndrome, the type of antibody, and the presence or absence of cancer, patients are classified as having a "definite" or "possible" PNS. Despite the presumed autoimmune etiology of PNS, the results of various forms of immunotherapy have been disappointing, with some exceptions. Rapid detection and immediate treatment of the underlying tumor appears to offer the best chance of stabilizing the patient and preventing further neurological deterioration.

This article has been cited by other articles:

				Y.-S. Zang, Q.-Y. Xiu, Z. Fang, B. Li, and T.-B. Xia Case Report: Dramatic Recovery of Lung Adenocarcinoma-Associated Dermatomyositis with Targeted Lung Cancer Therapy Alone Oncologist, January 1, 2008; 13(1): 79 - 81. [Abstract] [Full Text] [PDF]

				S. L. Khella, N. Souyah, and J. Dalmau THYMOMA, MYASTHENIA GRAVIS, ENCEPHALITIS, AND A NOVEL ANTICYTOPLASMIC NEURONAL ANTIBODY Neurology, September 18, 2007; 69(12): 1302 - 1303. [Full Text] [PDF]

				S. Rajappa, R. Digumarti, S. R. Immaneni, and M. Parage Primary Renal Lymphoma Presenting With Paraneoplastic Limbic Encephalitis J. Clin. Oncol., August 20, 2007; 25(24): 3783 - 3785. [Full Text] [PDF]