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Original Contribution
March 2010

Paraneoplastic Neurologic Syndrome in the PNS Euronetwork Database: A European Study From 20 Centers

Author Affiliations

Author Affiliations: Department of Neurology, Ospedale Ca’Foncello, Treviso (Drs Giometto and Vitaliani), and Clinical Epidemiology Laboratory, Mario Negri Institute of Pharmacological Research, Milan (Dr Bertolini), Italy; Department of Neurooncology, Ludwig Boltzmann Institute of Vienna, Vienna, Austria (Dr Grisold); Neurology Service, Hospital Clinic, and Institut d’Investigació Biomèdica August Pi i Sunyer, Barcelona, Spain (Dr Graus); and Centre de Référence Maladie Rare Syndromes Neurologiques Paranéoplastiques Hospices Civils de Lyon, Hôpital Neurologique, Bron, and Institut National de la Santé et de la Récherche Médicale, Université Lyon 1, Lyon (Dr Honnorat), France.

Arch Neurol. 2010;67(3):330-335. doi:10.1001/archneurol.2009.341

Background  Paraneoplastic neurologic syndrome (PNS) represents the remote effects of cancer on the nervous system. Diagnostic criteria for the syndrome were published by the PNS Euronetwork and form the basis of a database to collect standardized clinical data from patients with PNS.

Objectives  To analyze various types of PNS, frequent tumor and antibody associations, clinical characteristics of individual syndromes, and possible therapeutic and prognostic strategies.

Design  Prospective case series and database study.

Setting  Twenty European centers.

Patients  Patients were recruited from January 1, 2000, to December 31, 2008.

Main Outcome Measures  Based on diagnostic criteria published by the PNS Euronetwork consortium, clinical characteristics of classic PNS and several other less well-characterized syndromes associated with cancer were assessed.

Results  Data from 979 patients were analyzed, representing the largest PNS investigation to date. The findings elucidate the clinical evolution of paraneoplastic cerebellar syndrome according to the onconeural antibodies present, the heterogeneity and prognosis of dysautonomic disorders, and the clinical variability of paraneoplastic limbic encephalitis.

Conclusion  The study results confirm that PNS influences oncologic patient survival. Tumors are the main cause of death, but some types of PNS (such as dysautonomia) have a poorer prognosis than malignant neoplasms.