Paraneoplastic disorders of the central and peripheral nervous systems

Handb Clin Neurol. 2014;121:1159-79. doi: 10.1016/B978-0-7020-4088-7.00078-X.


Paraneoplatic neurologic syndromes (PNS) have been seminally defined as acute or subacute neurological syndromes resulting from nervous system dysfunction that is remote from the site of a malignant neoplasm or its metastases. However, in respect to our current understanding of their pathogenesis we may redefine these disorders as cancer-related dysimmune neurologic syndromes. We first deal with the epidemiology and the pathogenesis of PNS, then the different classic PNS are reviewed with clinical features according to the associated onconeuronal antibodies. Finally, therapeutic approaches are discussed.

Keywords: Lambert–Eaton myasthenic syndrome; Paraneoplastic neurologic syndrome; limbic encephalitis; neuronal cell surface antibody; onconeuronal antibody; opsoclonus myoclonus; small-cell lung cancer; subacute cerebellar ataxia; subacute neuronopathy.

Publication types

  • Review

MeSH terms

  • Animals
  • Central Nervous System Neoplasms / diagnosis
  • Central Nervous System Neoplasms / epidemiology
  • Central Nervous System Neoplasms / genetics
  • Central Nervous System Neoplasms / pathology*
  • Central Nervous System Neoplasms / therapy
  • Humans
  • Immunotherapy
  • Neoplasm Proteins / genetics
  • Neoplasm Proteins / metabolism
  • Paraneoplastic Syndromes, Nervous System / diagnosis
  • Paraneoplastic Syndromes, Nervous System / epidemiology
  • Paraneoplastic Syndromes, Nervous System / genetics
  • Paraneoplastic Syndromes, Nervous System / pathology*
  • Paraneoplastic Syndromes, Nervous System / therapy
  • Peripheral Nervous System Neoplasms / diagnosis
  • Peripheral Nervous System Neoplasms / epidemiology
  • Peripheral Nervous System Neoplasms / genetics
  • Peripheral Nervous System Neoplasms / pathology*
  • Peripheral Nervous System Neoplasms / therapy


  • Neoplasm Proteins