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Managing paraneoplastic neurological disorders.
Oncologist 2006; 11(3):292-305O

Abstract

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.

Authors+Show Affiliations

Erasmus University Medical Center, Rotterdam, The Netherlands.No affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

16549814

Citation

de Beukelaar, Janet W., and Peter A. Sillevis Smitt. "Managing Paraneoplastic Neurological Disorders." The Oncologist, vol. 11, no. 3, 2006, pp. 292-305.
de Beukelaar JW, Sillevis Smitt PA. Managing paraneoplastic neurological disorders. Oncologist. 2006;11(3):292-305.
de Beukelaar, J. W., & Sillevis Smitt, P. A. (2006). Managing paraneoplastic neurological disorders. The Oncologist, 11(3), pp. 292-305.
de Beukelaar JW, Sillevis Smitt PA. Managing Paraneoplastic Neurological Disorders. Oncologist. 2006;11(3):292-305. PubMed PMID: 16549814.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Managing paraneoplastic neurological disorders. AU - de Beukelaar,Janet W, AU - Sillevis Smitt,Peter A, PY - 2006/3/22/pubmed PY - 2006/8/12/medline PY - 2006/3/22/entrez SP - 292 EP - 305 JF - The oncologist JO - Oncologist VL - 11 IS - 3 N2 - 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. SN - 1083-7159 UR - https://www.unboundmedicine.com/medline/citation/16549814/Managing_paraneoplastic_neurological_disorders_ L2 - https://doi.org/10.1634/theoncologist.11-3-292 DB - PRIME DP - Unbound Medicine ER -