RT Journal Article
SR Electronic
T1 Paraneoplastic neurological syndromes: a practical approach to diagnosis and management
JF Practical Neurology
JO Pract Neurol
FD BMJ Publishing Group Ltd
SP practneurol-2021-003073
DO 10.1136/practneurol-2021-003073
A1 Sophie Binks
A1 Christopher Uy
A1 Jerome Honnorat
A1 Sarosh R Irani
YR 2021
UL http://pn.bmj.com/content/early/2021/09/10/practneurol-2021-003073.abstract
AB 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 helps 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.