Paraneoplastic Neurological Syndromes: Clinical And Serological Studies

Abstract

The term “paraneoplastic syndromes” refers to symptoms or signs resulting from damage to organs or tissues that are remote from the site of a malignant neoplasm or its metastases. Paraneoplastic syndromes can affect most organs and tissues including the nervous system. Since the fi rst time that the term “paraneoplastic” was used by Guichard and Vignon in 19491 reporting a case with polyradiculopathy and cancer of the uterus, the clinical presentation, associated tumors, the pathogenesis, diagnosis and management of paraneoplastic syndromes including the paraneoplastic neurological syndromes (PNS), has been continuously refi ned. However, the general concept described by Brain and Norris in 1965 for defi nition of PNS as a remote effect of cancer still applies . PNS are remote effects of cancer, i.e. not caused by invasion of the tumor or its metastases nor by any adverse event of cancer treatment and not by infection, metabolic disturbances or cerebrovascular complications. The true prevalence of PNS is not yet established and varies between 0.01 - 1 percent of cancer patients. However it is noteworthy to mention much higher frequencies of some syndromes including Lambert-Eaton myasthenic syndrome (LEMS) in 3% of patients with small-cell lung cancer (SCLC)3, myasthenia gravis in 15% of patients diagnosed with thymoma and a severe predominantly motor neuropathy in about 50% of patients with the osteosclerotic form of plasmacytoma