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.
This is a preview of subscription content, access via your institution.
Buy single article
Instant access to the full article PDF.
Tax calculation will be finalised during checkout.
Subscribe to journal
Immediate online access to all issues from 2019. Subscription will auto renew annually.
Tax calculation will be finalised during checkout.
Paraneoplastic neurological syndrome
Modified Rankin scale
Obsessive compulsive disorder
Small cell lung cancer
- Non SCLC:
Non small cell lung cancer
Systemic lupus erythematosus
Central nervous system
Magnetic resonance imaging
Cardoso F, Seppi K, Mair KJ, Wenning GK, Poewe W (2006) Seminar on choreas. Lancet Neurol 5:589–602
Albin RL, Bromberg MB, Penney JB, Knapp R (1988) Chorea and dystonia: a remote effect of carcinoma. Mov Disord 3:162–169
Heckmann J, Lang CJ, Druschky A, Claus D, Bartels O, Neundörfer B (1997) Chorea resulting from paraneoplastic encephalitis. Mov Disord 12:464–466
Batchelor TT, Platten M, Palmer-Toy DE, Hunter GJ, Lev MH, Dalmau J, Hochberg FH (1998) Chorea as a paraneoplastic complication of Hodgkin’s disease. J Neurooncol 36:185–190
Nuti A, Ceravolo R, Salvetti S, Gambaccini G, Bonuccelli U, Capochiani E (2000) Paraneoplastic choreic syndrome during non-Hodgkin’s lymphoma. Mov Disord 15:350–352
Tani T, Piao Y, Mori S, Ishihara N, Tanaka K, Wakabayashi K, Takahashi H (2000) Chorea resulting from paraneoplastic striatal encephalitis. J Neurol Neurosurg Psychiatry 69:512–551
Croteau D, Owainati A, Dalmau J, Rogers LR (2001) Response to cancer therapy in a patient with a paraneoplastic choreiform disorder. Neurology 57:719–722
Kujawa KA, Niemi VR, Tomasi MA, Mayer NW, Cochran E, Goetz CG (2001) Ballistic-choreic movements as the presenting feature of renal cancer. Arch Neurol 58:1133–1135
Vernino S, Tuite P, Adler CH, Meschia JF, Boeve BV, Boasberg P, Parigi JE, Lennon VA (2002) Paraneoplastic chorea associated with CRMP-5 neuronal antibody and lung carcinoma. Ann Neurol 51:625–630
Tremont-Lukats IW, Fuller GN, Ribalta T, Giglio P, Groves MD (2002) Paraneoplastic chorea: case study with autopsy confirmation. Neuro Oncol 4:192–195
Samii A, Dahlen DD, Spence AM, Maronian NC, Kraus EE, Lennon VA (2003) Paraneoplastic movement disorder in a patient with non-Hodgkin’s lymphoma and CRMP-5 autoantibody. Mov Disord 18:1556–1558
Kinirons P, Fulton A, Keoghan M, Brennan P, Farrell MA, Moroney JT (2003) Paraneoplastic limbic encephalitis (PLE) and chorea associated with CRMP-5 neuronal antibody. Neurology 61:1623–1624
Dorban S, Gille M, Kessler R, Piéret F, Declercq I, Sindic CJ (2004) Chorea-athetosis in the anti-Hu syndrome. Rev Neurol (Paris) 160:126–129
Muehlschlegel S, Okun MS, Foote KD, Coco D, Yachnis AT, Fernandez HH (2005) Paraneoplastic chorea with leukoencephalopathy presenting with obsessive-compulsive and behavioural disorder. Mov Disord 20:1523–1527
Krolak-Salmon P, Androdias G, Meyronet D, Aguera M, Honnorat J, Vighetto A (2006) Slow evolution of cerebellar degeneration and chorea in a man with anti-Yo antibodies. Eur J Neurol 13:307–308
Kellinghaus C, Kraus J, Blaes F, Nabavi DG, Schäbitz WR (2007) CRMP-5 autoantibodies in testicular cancer associated with limbic encephalitis and choreiform dyskinesias. Eur Neurol 57:241–243
Graus F, Delattre JY, Antoine JC, Dalmau J, Giometto B, Grisold W, Honnorat J, Smitt PS, Vedeler Ch, Verschuuren JJ, Vincent A, Voltz R (2004) Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 75:1135–1140
Willinson HJ, Ang W, Gilhus NE, Graus F, Liblau R, Vedeler C, Vincent A (2000) EFNS task force report: a questionnaire-based survey on the service provision and quality assurance for determination of diagnostic antibody test in European neuroimmunology centers. Eur J Neurol 7:625–628
Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J (2000) Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain 123:1481–1494
Graus F, Keime-Guibert F, Reñé R, Benyahia B, Ribalta T, Ascaso C, Escaramis G, Delattre JY (2001) Anti-Hu-associated paraneoplastic encephalomyelitis: analysis of 200 patients. Brain 124:1138–1148
Shams’ili S, Grefkens J, De Leeuw B, van den Bent M, Hooijkaas H, van der Holt B, Vecht C, Sillevis Smitt P (2003) Paraneoplastic cerebellar degeneration associated with antineuronal antibodies: analysis of 50 patients. Brain 126:1409–1418
Tüzün E, Dalmau J (2007) Limbic encephalitis and variants: classification, diagnosis and treatment. Neurologist 13:261–271
Jummani R, Okun M (2001) Sydenham chorea. Arch Neurol 58:311–313
Cervera R, Asherson RA, Font J, Cervera R, Asherson RA, Font J, Tikly M, Pallarés L, Chamorro A, Ingelmo M (1997) Chorea in the antiphospholipid syndrome. Clinical, radiologic, and immunologic characteristics of 50 patients from our clinics and the recent literature. Medicine (Baltimore) 76:203–212
Langbehn DR, Brinkman RR, Falush D, Paulsen JS, Hayden MR (2004) International Huntington’s Disease Collaborative Group. A new model for prediction of the age of onset and penetrance for Huntington’s disease based on CAG length. Clin Genet 65:267–277
Wild EJ, Tabrizi SJ (2007) The differential diagnosis of chorea. Pract Neurol 7:360–373
Dalmau J, Rosenfeld MR (2008) Paraneoplastic syndromes of the CNS. Lancet Neurol 7:327–340
Honnorat J, Cartalat-Carel S, Ricard D, Camdessanche JP, Carpentier AF, Rogemond V, Chapuis F, Aguera M, Decullier E, Duchemin AM, Graus F, Antoine JC (2009) Onco-neural antibodies and tumour type determine survival and neurological symptoms in paraneoplastic neurological syndromes with Hu or CV2/CRMP5 antibodies. J Neurol Neurosurg Psychiatry 80:412–416
Scheid R, Voltz R, Guthke T, Bauer J, Sammler D, von Cramon DY (2003) Neuropsychiatric findings in anti-Ma2-positive paraneoplastic limbic encephalitis. Neurology 61:1159–1161
Maia DP, Teixeira AL, Quintão Cunningham MC, Cardoso F (2005) Obsessive compulsive behavior, hyperactivity, and attention deficit disorder in Sydenham chorea. Neurology 64:1799–1801
Maina G, Albert U, Bogetto F, Borghese C, Berro AC, Mutani R, Rossi F, Vigliani MC (2009) Anti-brain antibodies in adult patients with obsessive-compulsive disorder. J Affect Disord 116:192–200
Dalmau J, Lancaster E, Martinez-Hernadez E, Rosenfeld MR, Balice-Gordon R (2011) Clinical experience and laboratory investigations in patients with NMDAR encephalitis. Lancet Neurol 10:63–79
Rankin J (1957) Cerebral vascular accidents in patients over the age of 60. II. Prognosis. Scott Med J 2:200–215
Psimaras D, Carpentier A, Rossi C, PNS Euronetwork (2010) CSF study in paraneoplastic syndrome. J Neurol Neurosurg Psychiatry 81:42–45
Saiz A, Bruna J, Stourac P, Vigliani MC, Giometto B, Grisold W, Honnorat J, Psimaras D, Voltz R, Graus F (2009) Anti-Hu-associated brainstem encephalitis. J Neurol Neurosurg Psychiatry 80:404–440
Rogemond V, Honnorat J (2000) Anti-CV2 autoantibodies and paraneoplastic neurological syndromes. Clin Rev Allergy Immunol 19:51–59
Honnorat J, Cartalat-Carel S (2004) Advances in paraneoplastic neurological syndromes. Curr Opin Oncol 16:614–620
Voltz R (2002) Paraneoplastic neurological syndromes: an update on diagnosis, pathogenesis, and therapy. Lancet Neurol 1:294–305
Rojas I, Graus F, Keime-Guibert F, Reñé R, Delattre JY, Ramón JM, Dalmau J, Posner J (2000) Long-term clinical outcome of paraneoplastic cerebellar degeneration and anti-Yo antibodies. Neurology 55:713–715
Sahashi K, Sakai K, Mano K, Hirose G (2003) Anti-Ma2 antibody related paraneoplastic limbic/brain stem encephalitis associated with breast cancer expressing Ma1, Ma2, and Ma3 mRNAs. J Neurol Neurosurg Psychiatry 74:1332–1335
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.
Conflict of interest
Members of PNS EuroNetwork are given in the Appendix.
Electronic supplementary material
Below is the link to the electronic supplementary material.
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.
About this article
Cite this article
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). https://doi.org/10.1007/s00415-011-6074-1
- Movement disorder