Neurological paraneoplastic syndromes: an update
Giulia Berzero1,2, Dimitri Psimaras3,4
1Neuroncology Unit, IRCCS Mondino Foundation.
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.
Insights
Recent advances in paraneoplastic neurological syndromes (PNS) highlight differences in treatment outcomes. Autoimmunity targeting cell-surface antigens (csPNS) shows better response to immunotherapy than intracellular antigen PNS (icPNS).
Area of Science:
- Neurology
- Immunology
- Oncology
Background:
- Paraneoplastic neurological syndromes (PNS) are rare cancer complications arising from immune cross-reactivity against neuronal antigens.
- PNS pathogenesis differs based on antibody targets: intracellular antigens (icPNS) vs. cell-surface antigens (csPNS).
- icPNS exhibit relentless progression and poor treatment response due to irreversible neuronal damage.
Purpose of the Study:
- To review recent diagnostic and therapeutic advancements in paraneoplastic neurological syndromes.
- To differentiate clinical profiles, pathogenesis, and outcomes of icPNS and csPNS.
- To emphasize the importance of optimizing immunotherapy for csPNS.
Main Methods:
- Review of current literature on paraneoplastic neurological syndromes.
- Analysis of diagnostic criteria and therapeutic strategies.
- Comparison of outcomes based on antibody targets (intracellular vs. cell-surface).
Main Results:
- The discovery of novel cell-surface antibodies has expanded the understanding of paraneoplastic autoimmunity.
- csPNS demonstrate a generally favorable response to immune therapy and better functional outcomes.
- icPNS are associated with severe neuronal loss and limited therapeutic efficacy.
Conclusions:
- The expanding spectrum of paraneoplastic autoimmunity necessitates updated diagnostic and treatment approaches.
- Optimizing immune therapies for csPNS is crucial for improving patient outcomes and preventing relapses.
- Distinguishing between icPNS and csPNS is vital for predicting prognosis and guiding treatment decisions.
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