title: Paraneoplastic neurological syndromes.
abstract: OBJECTIVE
  This review describes relevant advances in paraneoplastic neurological syndromes (PNS) with emphasis on particular syndromes and the impact of antibodies against surface antigens in their management.
  
  
  RESULTS
  PNS may present with symptoms that do not raise the suspicion of a paraneoplastic origin. The best example is anti-N-methyl-D-aspartate receptor encephalitis that in adult women frequently associates with ovarian teratoma. An electroencephalogram pattern described as 'extreme delta brush' was recently identified in 30% of patients with this disorder. Isolated myelopathy may have a paraneoplastic origin associated with amphiphysin or CV2 (CRMP5) antibodies. Jaw dystonia and laryngospasm can be the predominant symptom of the brainstem encephalitis associated with Ri antibodies. γ-Aminobutyric acid (GABA)(B) receptor antibodies are the most common antibodies found in patients with limbic encephalitis and small cell lung cancer, and contactin-associated protein 2 antibodies in patients with Morvan's syndrome and thymoma. Lastly, a recent study identified delta/notch-like epidermal growth factor-related receptor (DNER) as the target antigen of Tr antibodies, a marker of cerebellar ataxia and Hodgkin's lymphoma.
  
  
  CONCLUSIONS
  The number of antibodies relevant to PNS is now expanded to those against surface antigens. These antibodies do not confirm the paraneoplastic origin of the syndrome but predict a better response to immunotherapy.
authors.affiliation: Service of Neurology, Hospital Clinic, Universitat de Barcelona and Institut d'Investigació Biomèdica August Pi i Sunyer, Barcelona, Spain. fgraus@clinic.ub.es
authors.forename: Francesc
authors.initials: F
authors.lastname: Graus
authors.forename: Josep
authors.initials: J
authors.lastname: Dalmau
mesh_terms: D001323:Autoantibodies / Q000276:immunology*; D006801:Humans; D007167:Immunotherapy; D009369:Neoplasms / Q000150:complications*; D020361:Paraneoplastic Syndromes, Nervous System / Q000209:etiology / Q000276:immunology / Q000628:therapy
chemical_list: D001323:Autoantibodies