Paraneoplastic neurological syndromes: a practical approach to diagnosis and management
| Autor: | Sophie Binks, Jérôme Honnorat, Sarosh R. Irani, Christopher E Uy |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Epitope
Serology 03 medical and health sciences 0302 clinical medicine Antigen Medicine Humans Clinical significance 030304 developmental biology Autoantibodies 0303 health sciences biology business.industry Autoantibody Cancer General Medicine medicine.disease 3. Good health Neuroimmunology Immunology biology.protein Neurology (clinical) Immunotherapy Antibody business 030217 neurology & neurosurgery Paraneoplastic Syndromes Nervous System |
| Zdroj: | Practical neurology. 22(1) |
| ISSN: | 1474-7766 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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