Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience


Chorea and other movement disorders are rarely described as paraneoplastic. The aim of this study was to describe 13 patients with paraneoplastic chorea and dystonia collected by the members of the paraneoplastic neurological syndrome (PNS) EuroNetwork and to review 29 cases from the literature. We analyzed neurological symptoms, severity of the neurological syndrome, delay in neurological diagnosis, associated cancer, oncological and neurological treatments received, and outcome. Eleven (1.2%) out of 913 patients with PNS were identified in the EuroNetwork register. Two more patients not included in the register were added. The overall population consisted of 13 patients with a median age of 75 years (range 49–82 years). In most patients, the movement disorder was classical choreoathetosis with symmetric involvement of the trunk, neck, and limbs. A minority of patients presented unilateral chorea, dystonia, and orobuccal dyskinesia. Associated symptoms, as polyneuropathy, encephalitis, psychiatric disturbances, or visual defects, were often present. The movement disorder usually had a subacute course. The most frequently associated cancer was small cell lung cancer (SCLC). Lymphoma, bowel, or kidney cancers were also reported. CV2/CRMP5 was the most frequently associated antibody, followed by Hu. Hyperintense lesions of the basal ganglia on T2-weighted images were seldom observed. Response to cancer therapy was observed in a minority of patients, but survival was short (17 months). As in other neurological diseases, movement disorders should also be suspected as paraneoplastic when they develop subacutely in older patients (usually over 50) and often in the presence of other ancillary neurological symptoms.

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Fig. 1



Paraneoplastic neurological syndrome


Modified Rankin scale


Obsessive compulsive disorder


Small cell lung cancer


Non small cell lung cancer


Hodgkin's lymphoma


Non-Hodgkin's lymphoma


Systemic lupus erythematosus


Central nervous system


Cerebrospinal fluid


Magnetic resonance imaging


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We thank Ms. Joanne Fleming for her technical support. This study was supported by Grants QLG1-CT-2002-01756 and LSSM-CT-2005-518174 of the European Commission.

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Corresponding author

Correspondence to Maria Claudia Vigliani.

Additional information

Members of PNS EuroNetwork are given in the Appendix.

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Below is the link to the electronic supplementary material.

Video 1: Symmetric chorea in a patient CV2-positive developing a SCLC (Patient no. 7): 1 (WMV 1577 kb)



The PNS EuroNetwork is formed by: B. Giometto, Treviso, Italy; P. Tonali, Rome, Italy; M.C. Vigliani, Torino, Italy; J. Honnorat, Lyon, France; R. Voltz, Cologne, Germany; F. Graus, Barcelona, Spain; P. Stourac, Brno, Czech Republic; R. Fazio, Milan, Italy; P. Sillevis Smitt, Rotterdam, Netherlands; J. Verschuuren, Leiden, Netherlands; A. Carpentier, Paris, France; A. Vincent, Oxford, UK; J. C. Antoine, St. Etienne, France; J. Rees, London, UK; W. Grisold, Vienna, Austria; C. Vedeler, Bergen, Norway; R. Zoran, Ljubljana, Slovenia; (the late) I. Hart, Liverpool, UK; L. Lorusso, Chiari, Italy; G. Bertolini, Bergamo, Italy; G. Vita, Messina, Italy; F. Blaes, Giessen, Germany; S. Rauer, Freiburg, Germany.

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Vigliani, M.C., Honnorat, J., Antoine, JC. et al. Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience. J Neurol 258, 2058–2068 (2011).

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  • Paraneoplastic
  • Chorea
  • Dystonia
  • Movement disorder
  • Cancer