In this chapter we review the CNS syndromes mediated by autoimmune or inflammatory mechanisms in patients with cancer. Paraneoplastic neurological syndromes (PNS) are considered to be immune-mediated disorders against proteins expressed by the tumour and nervous system. The autoimmune hypothesis is supported by the presence in serum and CSF of antibodies against neural proteins that are also expressed in the tumour. Less frequently the tumour does not express neuronal proteins but predisposes to immune dysregulation and autoimmune mechanisms. Novel cancer therapies that enhance anti-tumour immune responses frequently cause inflammatory CNS disorders. Immune checkpoint inhibitors have been associated with a wide range of immune-related adverse effects, including an increased incidence of PNS. Another type of cancer therapy is based on the use of T cells genetically engineered to express chimeric antigen receptors (CARs) that recognize molecules present on the surface of tumour cells. CAR T cell therapy can cause severe, potentially lethal, encephalopathy syndromes mediated by massive release of cytokines instead of autoimmune mechanisms. Post-transplant autoimmune encephalitis are rare disorders that mostly occur after allogeneic haematopoietic stem cell transplantation. They are related to graft versus host disease and, sometimes, they associate with antibodies against neuronal surface antigens.

In this chapter we review the CNS syndromes mediated by autoimmune or inflammatory mechanisms in patients with cancer. Paraneoplastic neurological syndromes (PNS) are considered to be immune-mediated disorders against proteins expressed by the tumour and nervous system. The autoimmune hypothesis is supported by the presence in serum and CSF of antibodies against neural proteins that are also expressed in the tumour. Less frequently the tumour does not express neuronal proteins but predisposes to immune dysregulation and autoimmune mechanisms. Novel cancer therapies that enhance anti-tumour immune responses frequently cause inflammatory CNS disorders. Immune checkpoint inhibitors have been associated with a wide range of immune-related adverse effects, including an increased incidence of PNS. Another type of cancer therapy is based on the use of T cells genetically engineered to express chimeric antigen receptors (CARs) that recognize molecules present on the surface of tumour cells. CAR T cell therapy can cause severe, potentially lethal, encephalopathy syndromes mediated by massive release of cytokines instead of autoimmune mechanisms. Post-transplant autoimmune encephalitis are rare disorders that mostly occur after allogeneic haematopoietic stem cell transplantation. They are related to graft versus host disease and, sometimes, they associate with antibodies against neuronal surface antigens.