Paraneoplastic disorders of the nervous system

Abstract

This article on paraneoplastic neurologic disorders provides an update on the diagnostic approach, utility and pitfalls of autoantibody testing and emerging settings in which these disorders are encountered. Recognition of the clinical and neuroimaging features accompanying paraneoplastic neurologic disorders is crucial to select those at highest risk who need neural antibody testing and screening for cancer. Cursory knowledge of the antibody assay methodology being ordered is important as the false positive rate varies by the technique utilized for detection. Antibodies can generally be stratified by the location of the target antigen (intraceullar versus cell-surface/synaptic) which informs frequency of cancer association, treatment response and prognosis. The therapeutic approach generally involves detection of the underlying cancer and combinations of oncologic treatments and immunosuppressant medications. The occurrence of paraneoplastic autoimmune neurologic disorders in novel settings, such as with immune checkpoint inhibitor use, has improved understanding of their pathogenesis and increased the likelihood neurologists will encounter such patients in their practice.