Skip to main content
SpringerLink
Log in

Paraneoplastic Neurological Syndromes, Overview

  • Reference work entry
  • First Online:
Encyclopedia of Medical Immunology

  • 606 Accesses

Definition

Paraneoplastic neurological syndromes (PNS) are a group of disorders resulting from damage to the nervous system in the setting of cancer, but remote from the site of cancer, and not related to metastasis, infection, or metabolic derangements otherwise associated with cancer. They result from direct damage to neural tissue through an immune-mediated mechanism itself triggered either by an immunologic disturbance caused by the tumor or by tumors that express neuronal proteins. PNS may affect either the central or peripheral nervous system or both, and they may be isolated to a single neuronal cell type or site or affect more diffuse involvement of the nervous system. Antineuronal antibodies, useful as diagnostic markers of PNS and the underlying cancer, are often associated with these immune responses, some being strongly associated with specific tumor types, some less so (Graus et al. 2004). Several recognizable clinical syndromes have been identified (Table 1) and will be...

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 799.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 949.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Bataller L, Dalmau J. Paraneoplastic neurologic syndromes: approaches to diagnosis and treatment. Sem in Neurol. 2003;23(2):215–24.

    Google Scholar 

  • Darnell RB. Paraneoplastic neurologic disorders. Arch Neurol. 2004;61:30–2.

    PubMed  Google Scholar 

  • Darnell RB, DeAngelis LM. Regression of small-cell lung carcinoma in patients with paraneoplastic neuronal antibodies. Lancet. 1993;341:21–2.

    PubMed  CAS  Google Scholar 

  • Darnell RB, Posner JB. Paraneoplastic syndromes involving the nervous system. N Eng J Med. 2003;349:1543–54.

    CAS  Google Scholar 

  • Darnell RB, Posner JB. Paraneoplastic syndromes affecting the nervous system. Semin Oncol. 2006;33:270–98.

    PubMed  Google Scholar 

  • Fuller CE, Narendra S. Paraneoplastic disorders of the central nervous system. In: Adesina AM et al., editors. Atlas of pediatric brain tumors. doi:10.1007/978-1-4419-1062-2_34, © Springer Science + Business Media, LLC 2010.

    Google Scholar 

  • Graus F, Delattre JY, Antoine JC, et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry. 2004;75:1135–40.

    PubMed  CAS  PubMed Central  Google Scholar 

  • Graus F, Florence KG, Reñé R, et al. Anti-Hu associated paraneoplastic encephalomyelitis: analysis of 200 patients. Brain. 2001;124:1138–48.

    PubMed  CAS  Google Scholar 

  • Greenlee JE. Recommended diagnostic criteria for paraneoplastic neurological syndromes: editorial commentary. J Neurol Neurosurg Psychiatry. 2004;75:1090.

    PubMed  CAS  PubMed Central  Google Scholar 

  • Honnorat J, Antoine JC. Paraneoplastic neurological syndromes. Orphanet J Rare Dis. 2007;2:22.

    PubMed  PubMed Central  Google Scholar 

  • Kim YI. Passively transferred Lambert-Eaton syndrome in mice receiving purified IgG. Muscle Nerve. 1986;9:523–30.

    PubMed  CAS  Google Scholar 

  • Latov N. Pathogenesis and therapy of neuropathies associated with monoclonal gammopathies. Ann Neurol. 1995;37(S1):S32–42.

    PubMed  Google Scholar 

  • Lawn ND, Westmoreland BF, Kiely MJ, et al. Clinical, magnetic resonance imaging, and electroencephalographic findings in paraneoplastic limbic encephalitis. Mayo Clin Proc. 2003;78:1363–8.

    PubMed  Google Scholar 

  • Musunuru K, Darnell RB. Paraneoplastic neurologic disease antigens. Annu Rev Neurosci. 2001;24:239–62.

    PubMed  CAS  Google Scholar 

  • Pottock SJ, Kryzer TJ, Lennon VA. Paraneoplastic antibodies coexist and predict cancer, not neurological syndrome. Ann Neurol. 2004;56:715–9.

    Google Scholar 

  • Raspotnig M, Vedeler CA, Storstein A. Onconeural antibodies in patients with neurological symptoms: detection and clinical significance. Acta Neurol Scand. 2011;124(Suppl. 191):83–8.

    Google Scholar 

  • Roberts WK, Darnell RB. Neuroimmunology of the paraneoplastic neurological degenerations. Curr Opin Immunol. 2004;16:616–22.

    PubMed  CAS  Google Scholar 

  • Suete T, Leffers P, Ten Velde GP, et al. Neurologic disorders in 432 consecutive patients with small cell lung carcinoma. Cancer. 2004;100:801–6.

    Google Scholar 

  • Tanaka K, Ding X, Tanaka M. Effects of antineuronal antibodies from patients with paraneoplastic neurological syndrome on primary cultured neurons. J Neurol Sci. 2004;217:25–30.

    PubMed  CAS  Google Scholar 

  • Titulaer MJ, Soffietti R, Dalmau J, et al. Screening for tumours in paraneoplastic syndromes: report of an EFNS Task Force. Eur J Neurol. 2011;18:19–27.

    PubMed  CAS  PubMed Central  Google Scholar 

  • Vernino S, Lennon VA. Autoantibody profiles and neurological correlations of thymoma. Clin Cancer Res. 2004;10:7270–5.

    PubMed  CAS  Google Scholar 

  • Younes-Mhenni S, Janie MF, Cinotti L, et al. FDG-PET improves tumor detection in patients with paraneoplastic neurological syndromes. Brain. 2004;127:2331–8.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Weill Cornell Medical College, 520 East 70th Street, Rm K-615, New York, NY, 10021, USA

    Michael Rubin

  2. New York Medical College, 40 Sunshine Cottage Rd, Valhalla, NY, 10595, USA

    Jason Broker

  3. Associated Neurologist of Southern Connecticut, Fairfield, CT, USA

    Thomas B. Toothaker

Authors
  1. Michael Rubin
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jason Broker
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Thomas B. Toothaker
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Michael Rubin .

Editor information

Editors and Affiliations

  1. Department of Biochemistry and Molecular Biology, Monash University, Clayton, Victoria, Australia

    Ian R. Mackay

  2. Center for Autoimmune Disease Research, Department of Molecular Microbiology and Immunology, Department of Pathology, Johns Hopkins University, Baltimore, MD, USA

    Noel R. Rose

  3. The Feinstein Institute for Medical Research, Manhasset, NY, USA

    Betty Diamond

  4. The Feinstein Institute for Medical Research, Manhasset, NY, USA

    Anne Davidson

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer Science+Business Media New York

About this entry

Cite this entry

Rubin, M., Broker, J., Toothaker, T.B. (2014). Paraneoplastic Neurological Syndromes, Overview. In: Mackay, I.R., Rose, N.R., Diamond, B., Davidson, A. (eds) Encyclopedia of Medical Immunology. Springer, New York, NY. https://doi.org/10.1007/978-0-387-84828-0_5

Download citation

  • DOI: https://doi.org/10.1007/978-0-387-84828-0_5

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-84827-3

  • Online ISBN: 978-0-387-84828-0

  • eBook Packages: MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation