Update on paraneoplastic neurological syndromes

To describe specificities and new advances in treatment of paraneoplastic neurological syndromes (PNS).

PNS are defined as neurological syndromes of unknown cause that often antedate the diagnosis of an underlying cancer that is usually not clinically evident. The clinical signs of PNS are accurately described and 'classical' PNS have been established. Within the context of these syndromes, the concept of limbic encephalitis has evolved dramatically in the last 2 years due to the description of new auto-antibodies. The diagnosis and treatment of the associated cancer remains the key goal of the clinical management of PNS. However, the specific treatment of the neurological symptoms primarily depends on the immunological findings.

There is increasing recognition of an extensive array of PNS and of several paraneoplastic antibodies as biological markers of these disorders. Basic immunological studies support the pathogenic role of some of these antibodies. Others are only markers of the disease.