The review presents an overview on the pathogenesis of paraneoplastic
neurological disorders (PNDs) and the current therapeutic immunosuppressive or immunomodulatory strategies used in these patients. PNDs are disturbances in the functioning of the nervous system in
cancer patients, where the disturbances are not due to a local effect of the tumour or its
metastases. Most of these clinically, well-defined syndromes in adults are associated with
lung cancer (especially
small cell lung cancer),
lymphomas and gynaecological tumours. Since
autoantibodies directed against
proteins expressed in neurons and tumour cells have been found, PNDs are suspected to be autoimmune. In neuromuscular PND, immunosuppressive therapies,
plasmapheresis and
intravenous immunoglobulins are effective treatments. In contrast, central nervous system PNDs seen in adults are by far the most problematic group to treat. With exception of the
stiff-man syndrome, immunosuppression appears to have little effect on these
neurological disorders. Tumour
therapy stabilises PNDs but does not cause improvement.
Plasmapheresis reduces the
autoantibody titre in the sera of these patients but, like tumour
therapy, does not lead to a clinical improvement. In children with paraneoplastic
opsoclonus-myoclonus syndrome,
steroids and
intravenous immunoglobulins may lead to a complete or partial remission of PNDs.