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Recommended diagnostic criteria for paraneoplastic neurological syndromes
  1. F Graus1,
  2. J Y Delattre2,
  3. J C Antoine3,
  4. J Dalmau4,
  5. B Giometto5,
  6. W Grisold6,
  7. J Honnorat7,
  8. P Sillevis Smitt8,
  9. Ch Vedeler9,
  10. J J G M Verschuuren10,
  11. A Vincent11,
  12. R Voltz12,
  13. for the Paraneoplastic Neurological Syndrome Euronetwork
  1. 1Service of Neurology, Institut d’Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Hospital Clínic, University of Barcelona. Barcelona, Spain
  2. 2Service of Neurology, Hôpital de la Salpêtrière, Unité Inserm 495, Paris, France
  3. 3Department of Neurology, Hôpital Bellevue, Saint Etienne, France
  4. 4Department of Neurology, University of Pennsylvania, Philadelphia, USA
  5. 5Department of Neurology and Psychiatry (2 Neurologic Clinic), University of Padua, Padua, Italy
  6. 6Ludwig Boltzmann Institut für Neuroonkologie, Vienna, Linz, Austria
  7. 7Ataxia Research Center, Neurology B, Hôpital Neurologique, Lyon, France
  8. 8Department of Neurology, Erasmus University Medical Center, Rotterdam, The Netherlands
  9. 9Department of Neurology, Haukeland University Hospital, Bergen, Norway
  10. 10Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands
  11. 11Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK
  12. 12Institute of Clinical Neuroimmunology. University of Munich, Munich, Germany
  1. Correspondence to:
 Dr F Graus
 Servei de Neurologia, Hospital Clínic. Villarroel 170, Barcelona 08036, Spain; grausmedicina.ub.es

Abstract

Background: Paraneoplastic neurological syndromes (PNS) are defined by the presence of cancer and exclusion of other known causes of the neurological symptoms, but this criterion does not separate “true” PNS from neurological syndromes that are coincidental with a cancer.

Objective: To provide more rigorous diagnostic criteria for PNS.

Methods: An international panel of neurologists interested in PNS identified those defined as “classical” in previous studies. The panel reviewed the existing diagnostic criteria and recommended new criteria for those in whom no clinical consensus was reached in the past. The panel reviewed all reported onconeural antibodies and established the conditions to identify those that would be labelled as “well characterised”. The antibody information was obtained from published work and from unpublished data from the different laboratories involved in the study.

Results: The panel suggest two levels of evidence to define a neurological syndrome as paraneoplastic: “definite” and “possible”. Each level can be reached combining a set of criteria based on the presence or absence of cancer and the definitions of “classical” syndrome and “well characterised” onconeural antibody.

Conclusions: The proposed criteria should help clinicians in the classification of their patients and the prospective and retrospective analysis of PNS cases.

  • LEMS, Lambert-Eaton myasthenic syndrome
  • PNS, paraneoplastic neurological syndrome
  • VGCC, voltage gated calcium channel
  • autoantibodies
  • nervous system
  • paraneoplasia
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