Article Text
Abstract
Paraneoplastic neurological syndromes (PNS) are the immune-mediated effects of a remote cancer and are characterised by an autoantibody response against antigens expressed by the tumour. Classically, well-characterised ‘onconeuronal’ antibodies target intracellular antigens and hence cannot access their antigens across intact cell membranes. The pathogenic mediators are likely to be neuronal-specific T cells. There is a variable response to immunotherapies and the clinical syndrome helps to direct the search for a specific set of tumours. By contrast, many newly emerging autoantibodies with oncological associations target cell surface epitopes and can exert direct pathogenic effects on both the central and peripheral nervous systems. Patients with these cell-surface directed autoantibodies often clearly respond to immunotherapies. Overall, the clinical, serological and oncological features in an individual patient help to determine the clinical relevance of the syndrome and hence guide its management. We summarise current knowledge and a practical approach to the investigation, diagnosis, treatment and outcomes of patients with suspected PNS.
- immunology
- tumours
- neuroimmunology
- paraneoplastic syndrome
- clinical neurology
Statistics from Altmetric.com
Other content recommended for you
- Which antibody and which cancer in which paraneoplastic syndromes?
- Republished: Which antibody and which cancer in which paraneoplastic syndromes?
- Recommended diagnostic criteria for paraneoplastic neurological syndromes
- Central nervous system neuronal surface antibody associated syndromes: review and guidelines for recognition
- Paraneoplastic syndromes: when to suspect, how to confirm, and how to manage
- Delayed onset of a second paraneoplastic neurological syndrome in eight patients
- Rhombencephalomyelitis due to possible paraneoplastic syndrome associated with Hodgkin’s lymphoma
- Paraneoplastic encephalopathy: an unusual presenting feature of bladder cancer metastasis
- Paraneoplastic cerebellar degeneration diagnosed by anti-Yo determination in a young woman with early breast cancer
- Treatment of paraneoplastic neurological syndromes with antineuronal antibodies (Anti-Hu, Anti-Yo) with a combination of immunoglobulins, cyclophosphamide, and methylprednisolone