The term paraneoplastic syndrome is now mainly reserved for non-metastatic manifestations of cancer that are considered to have some immunological explanation, i.e. they are not due to vascular disease, coagulopathy, infection, metabolic disorder, nutritional deficeincy or the complications of treatment. The frequency of neurological paraneoplastic syndromes is something less than 0.5 per 100 000 population per year. Over the last 10 years, there has been increasing understanding of their immunological mechanisms and many new antigens/antibodies have been described. It is likely that the next few years will see the description of even more and so the number of ‘antibody negative’ cases will continue to shrink.
The central theory of pathogenesis is that the syndromes are autoimmune in origin. The individual patient mounts an antibody response to one or more of their tumour antigens, and the antibodies produced cross react with molecular domains normally expressed on neurones. (Fig. 1).
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