To describe recent advances in the diagnosis and treatment of paraneoplastic neurological syndromes (PNS).
PNS are rare complications of cancer caused by an immune cross-reaction between antigens expressed by tumor cells and neurons. The target of the immune attack can be an intracellular antigen or a cell-surface antigen. Although both types of autoimmunity are ‘paraneoplastic’, as indirectly triggered by the presence of a tumor, they profoundly differ in terms of clinical profile, pathogenesis and outcome. PNS associated with antibodies to intracellular antigens (icPNS) are characterized by relentless progression and poor response to treatment, because of rapid and permanent neuronal loss. PNS associated with antibodies to cell-surface antigens (csPNS) generally show favorable response to immune therapy and good functional outcome, as they result from reversible neuronal dysfunction.
The spectrum of paraneoplastic autoimmunity has dramatically expanded following the discovery of cell-surface antibodies. Novel antibodies are incessantly discovered, some of which have a solid association with cancer. As csPNS usually respond to immune therapy, the optimization of current treatment strategies should have high priority to improve therapeutic results and prevent relapses.
aNeuroncology Unit, IRCCS Mondino Foundation
bPhD Program in Biomedical Sciences, University of Pavia, Pavia, Italy
cService de Neurologie 2-Mazarin, Hôpitaux Universitaires La Pitié Salpêtrière - Charles Foix, APHP
dInserm U 1127, CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonnes Universités, Paris, France
Correspondence to Giulia Berzero, Neuroncology Unit, IRCCS Mondino Foundation, Via Mondino, 2 - 27100 Pavia, Italy. Tel: +39 (0)382 380420; fax: +39 (0)382 380226; e-mail: firstname.lastname@example.org
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