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

Journal of Neurology volume 258, pages2058–2068(2011)Cite this article

Abstract

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.

Access options

Buy single article

Instant access to the full article PDF.

US$ 39.95

Tax calculation will be finalised during checkout.

Fig. 1

Abbreviations

PNS:

Paraneoplastic neurological syndrome

MRS:

Modified Rankin scale

OCD:

Obsessive compulsive disorder

SCLC:

Small cell lung cancer

Non SCLC:

Non small cell lung cancer

HL:

Hodgkin's lymphoma

Non-HL:

Non-Hodgkin's lymphoma

SLE:

Systemic lupus erythematosus

CNS:

Central nervous system

CSF:

Cerebrospinal fluid

MRI:

Magnetic resonance imaging

References

  1. 1.

    Cardoso F, Seppi K, Mair KJ, Wenning GK, Poewe W (2006) Seminar on choreas. Lancet Neurol 5:589–602

    PubMed  Article  Google Scholar 

  2. 2.

    Albin RL, Bromberg MB, Penney JB, Knapp R (1988) Chorea and dystonia: a remote effect of carcinoma. Mov Disord 3:162–169

    PubMed  Article  CAS  Google Scholar 

  3. 3.

    Heckmann J, Lang CJ, Druschky A, Claus D, Bartels O, Neundörfer B (1997) Chorea resulting from paraneoplastic encephalitis. Mov Disord 12:464–466

    PubMed  Article  CAS  Google Scholar 

  4. 4.

    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

    PubMed  Article  CAS  Google Scholar 

  5. 5.

    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

    PubMed  Article  CAS  Google Scholar 

  6. 6.

    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

    PubMed  Article  CAS  Google Scholar 

  7. 7.

    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

    PubMed  CAS  Google Scholar 

  8. 8.

    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

    PubMed  Article  CAS  Google Scholar 

  9. 9.

    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

    PubMed  Article  Google Scholar 

  10. 10.

    Tremont-Lukats IW, Fuller GN, Ribalta T, Giglio P, Groves MD (2002) Paraneoplastic chorea: case study with autopsy confirmation. Neuro Oncol 4:192–195

    PubMed  Google Scholar 

  11. 11.

    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

    PubMed  Article  Google Scholar 

  12. 12.

    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

    PubMed  CAS  Google Scholar 

  13. 13.

    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

    Article  CAS  Google Scholar 

  14. 14.

    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

    PubMed  Article  Google Scholar 

  15. 15.

    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

    PubMed  Article  CAS  Google Scholar 

  16. 16.

    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

    PubMed  Article  Google Scholar 

  17. 17.

    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

    PubMed  Article  CAS  Google Scholar 

  18. 18.

    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

    Article  Google Scholar 

  19. 19.

    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

    PubMed  Article  Google Scholar 

  20. 20.

    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

    PubMed  Article  CAS  Google Scholar 

  21. 21.

    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

    PubMed  Article  Google Scholar 

  22. 22.

    Tüzün E, Dalmau J (2007) Limbic encephalitis and variants: classification, diagnosis and treatment. Neurologist 13:261–271

    PubMed  Article  Google Scholar 

  23. 23.

    Jummani R, Okun M (2001) Sydenham chorea. Arch Neurol 58:311–313

    PubMed  Article  CAS  Google Scholar 

  24. 24.

    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

    Article  CAS  Google Scholar 

  25. 25.

    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

    PubMed  Article  CAS  Google Scholar 

  26. 26.

    Wild EJ, Tabrizi SJ (2007) The differential diagnosis of chorea. Pract Neurol 7:360–373

    PubMed  Article  CAS  Google Scholar 

  27. 27.

    Dalmau J, Rosenfeld MR (2008) Paraneoplastic syndromes of the CNS. Lancet Neurol 7:327–340

    PubMed  Article  Google Scholar 

  28. 28.

    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

    PubMed  Article  CAS  Google Scholar 

  29. 29.

    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

    PubMed  CAS  Google Scholar 

  30. 30.

    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

    PubMed  Article  Google Scholar 

  31. 31.

    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

    PubMed  Article  CAS  Google Scholar 

  32. 32.

    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

    PubMed  Article  CAS  Google Scholar 

  33. 33.

    Rankin J (1957) Cerebral vascular accidents in patients over the age of 60. II. Prognosis. Scott Med J 2:200–215

    PubMed  CAS  Google Scholar 

  34. 34.

    Psimaras D, Carpentier A, Rossi C, PNS Euronetwork (2010) CSF study in paraneoplastic syndrome. J Neurol Neurosurg Psychiatry 81:42–45

    PubMed  Article  CAS  Google Scholar 

  35. 35.

    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

    PubMed  Article  CAS  Google Scholar 

  36. 36.

    Rogemond V, Honnorat J (2000) Anti-CV2 autoantibodies and paraneoplastic neurological syndromes. Clin Rev Allergy Immunol 19:51–59

    PubMed  Article  CAS  Google Scholar 

  37. 37.

    Honnorat J, Cartalat-Carel S (2004) Advances in paraneoplastic neurological syndromes. Curr Opin Oncol 16:614–620

    PubMed  Article  Google Scholar 

  38. 38.

    Voltz R (2002) Paraneoplastic neurological syndromes: an update on diagnosis, pathogenesis, and therapy. Lancet Neurol 1:294–305

    PubMed  Article  Google Scholar 

  39. 39.

    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

    PubMed  CAS  Google Scholar 

  40. 40.

    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

    PubMed  Article  CAS  Google Scholar 

Download references

Acknowledgments

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

None.

Author information

Affiliations

  1. Dipartimento di Neuroscienze, Ospedale San Giovanni Battista, Via Cherasco 15, 10126, Torino, Italy

    Maria Claudia Vigliani & Federica Franchino

  2. Hospices Civils de Lyon, Hôpital Neurologique, Centre de Référence Maladie Rare “Syndromes Neurologiques Paranéoplasiques”, Lyon, France

    Jerome Honnorat & Jean-Christophe Antoine

  3. INSERM, U842, Lyon; Université Lyon1, UMR-S842, 69008, Lyon, France

    Jerome Honnorat & Jean-Christophe Antoine

  4. Service de Neurologie, CHU, et Université de Saint-Etienne, 42100, Saint-Etienne, France

    Jean-Christophe Antoine

  5. Dipartimento di Neurologia, Ospedale Ca’ Foncello, Treviso, Italy

    Roberta Vitaliani & Bruno Giometto

  6. Division Mazarin, Hôpital de la Salpetrière, Paris, France

    Dimitri Psimaras

  7. Istituto Mario Negri, Bergamo, Italy

    Carlotta Rossi

  8. Department of Neurology, Hospital Clinic, Universitat de Barcelona and Institut d’Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Barcelona, Spain

    Francesc Graus

Authors
  1. Maria Claudia Vigliani
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jerome Honnorat
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jean-Christophe Antoine
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Roberta Vitaliani
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Bruno Giometto
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Dimitri Psimaras
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Federica Franchino
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Carlotta Rossi
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Francesc Graus
    View author publications

    You can also search for this author in PubMed Google Scholar

Consortia

and the PNS EuroNetwork

Corresponding author

Correspondence to Maria Claudia Vigliani.

Additional information

Members of PNS EuroNetwork are given in the Appendix.

Electronic supplementary material

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)

Appendix

Appendix

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.

Rights and permissions

Reprints and Permissions

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

Download citation

Keywords

  • Paraneoplastic
  • Chorea
  • Dystonia
  • Movement disorder
  • Cancer