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 19
OP 31
DO 10.1136/practneurol-2021-003073
VO 22
IS 1
A1 Binks, Sophie
A1 Uy, Christopher
A1 Honnorat, Jerome
A1 Irani, Sarosh R
YR 2022
UL http://pn.bmj.com/content/22/1/19.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 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.