Paraneoplastic epilepsy

Epilepsy Behav. 2016 Aug:61:51-58. doi: 10.1016/j.yebeh.2016.04.046. Epub 2016 Jun 12.


Epilepsy can be a manifestation of paraneoplastic syndromes which are the consequence of an immune reaction to neuronal elements driven by an underlying malignancy affecting other organs and tissues. The antibodies commonly found in paraneoplastic encephalitis can be divided into two main groups depending on the target antigen: 1) antibodies against neuronal cell surface antigens, such as against neurotransmitter (N-methyl-d-aspartate (NMDA), alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA), gamma-aminobutyric acid (GABA)) receptors, ion channels (voltage-gated potassium channel (VGKC)), and channel-complex proteins (leucine rich, glioma inactivated-1 glycoprotein (LGI1) and contactin-associated protein-2 (CASPR2)) and 2) antibodies against intracellular neuronal antigens (Hu/antineuronal nuclear antibody-1 (ANNA-1), Ma2/Ta, glutamate decarboxylase 65 (GAD65), less frequently to CV2/collapsin response mediator protein 5 (CRMP5)). In this review, we provide a comprehensive survey of the current literature on paraneoplastic epilepsy indexed by the associated onconeuronal antibodies. While a range of seizure types can be seen with paraneoplastic syndromes, temporal lobe epilepsy is the most common because of the association with limbic encephalitis. Early treatment of the paraneoplastic syndrome with immune modulation/suppression may prevent the more serious potential consequences of paraneoplastic epilepsy.

Keywords: Autoimmune epilepsy; Medically intractable epilepsy; Onconeuronal antibody; Paraneoplastic encephalitis; Paraneoplastic neurological syndrome.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases
  • Encephalitis / complications
  • Epilepsy / etiology*
  • Epilepsy / immunology
  • Epilepsy / therapy*
  • Humans
  • Paraneoplastic Syndromes, Nervous System / complications*
  • Paraneoplastic Syndromes, Nervous System / immunology
  • Paraneoplastic Syndromes, Nervous System / therapy*
  • Potassium Channels, Voltage-Gated
  • Prognosis


  • Potassium Channels, Voltage-Gated