Giulia Berzero1,2, Dimitri Psimaras3,4. 1. Neuroncology Unit, IRCCS Mondino Foundation. 2. PhD Program in Biomedical Sciences, University of Pavia, Pavia, Italy. 3. Service de Neurologie 2-Mazarin, Hôpitaux Universitaires La Pitié Salpêtrière - Charles Foix, APHP. 4. Inserm U 1127, CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonnes Universités, Paris, France.
Abstract
PURPOSE OF REVIEW: To describe recent advances in the diagnosis and treatment of paraneoplastic neurological syndromes (PNS). RECENT FINDINGS: 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. SUMMARY: 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.
PURPOSE OF REVIEW: To describe recent advances in the diagnosis and treatment of paraneoplastic neurological syndromes (PNS). RECENT FINDINGS: 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. SUMMARY: 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.
Authors: Anna E M Bastiaansen; Adriaan H C de Jongste; Marienke A A M de Bruijn; Yvette S Crijnen; Marco W J Schreurs; Marcel M Verbeek; Daphne W Dumoulin; Walter Taal; Maarten J Titulaer; Peter A E Sillevis Smitt Journal: Neurooncol Adv Date: 2021-09-28