Paraneoplastic Diseases of the Central Nervous System

F1000Res. 2020 Mar 6;9:F1000 Faculty Rev-167. doi: 10.12688/f1000research.21309.1. eCollection 2020.


Paraneoplastic neurological syndromes are nonmetastatic complications of malignancy secondary to immune-mediated neuronal dysfunction or death. Pathogenesis may occur from cell surface binding of antineuronal antibodies leading to dysfunction of the target protein, or from antibodies binding against intracellular antigens which ultimately leads to cell death. There are several classical neurological paraneoplastic phenotypes including subacute cerebellar degeneration, limbic encephalitis, encephalomyelitis, and dorsal sensory neuropathy. The patient's clinical presentations may be suggestive to the treating clinician as to the specific underlying paraneoplastic antibody. Specific antibodies often correlate with the specific underlying tumor type, and malignancy screening is essential in all patients with paraneoplastic neurological disease. Prompt initiation of immunotherapy is essential in the treatment of patients with paraneoplastic neurological disease, often more effective in cell surface antibodies in comparison to intracellular antibodies, as is removal of the underlying tumor.

Keywords: antibody-mediated; immunotherapy; neurological; paraneoplastic.

Publication types

  • Research Support, U.S. Gov't, Non-P.H.S.
  • Review

MeSH terms

  • Antibodies / analysis*
  • Central Nervous System Diseases / etiology*
  • Cerebellar Diseases / etiology
  • Humans
  • Immunotherapy
  • Neoplasms / complications*
  • Nerve Degeneration / etiology


  • Antibodies

Grants and funding

This research was supported by a Merit Review award from the US Department of Veterans Affairs (JEG).