The neuropathology of paraneoplastic syndromes

The term "paraneoplastic neurological syndromes" encompasses a number of un common disorders associated with systemic malignancies. In order to be clas sified a paraneoplastic neurological syndrome, the malignancies must not in vade, compress, or metastasize to the nervous system, They can either focal ly or diffusely involve the central and peripheral nervous system or the ne uromuscular junction. This paper reviews the neuropathology of the syndrome. It will first descri be the clinical presentation and give an account of the systemic tumors mos t commonly associated with the various types of disorders. Then it will rev iew the general pathological features that consist of an inflammatory proce ss predominantly affecting the gray matter. Finally, it will describe in de tail the main clinico-pathological types, including 1) encephalomyelitis, 2 ) cortical cerebellar degeneration, 3) peripheral neuropathy, 4) opsoclonus -myoclonus and 5) retinopathy, The Lambert-Eaton myasthenic syndrome will b e dealt with separately in another paper in this symposium.