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Onco-neural antibodies and tumour type determine survival and neurological symptoms in paraneoplastic neurological syndromes with Hu or CV2/CRMP5 antibodies
  1. J Honnorat1,2,3,
  2. S Cartalat-Carel1,
  3. D Ricard4,
  4. J Ph Camdessanche1,2,5,
  5. A F Carpentier1,6,
  6. V Rogemond1,2,
  7. F Chapuis3,7,
  8. M Aguera2,
  9. E Decullier3,7,
  10. A M Duchemin8,
  11. F Graus9,
  12. J C Antoine1,2,4
  1. 1
    Centre de Référence Maladies Rares “Syndromes Neurologiques Paranéoplasiques,” Hospices Civils de Lyon, Hôpital Neurologique, Bron, France
  2. 2
    UMR INSERM S842, Lyon, France
  3. 3
    Université de Lyon, Université Lyon 1, Lyon, France
  4. 4
    Hôpital du Val de Grâce, Neurologie, Paris, France
  5. 5
    Hôpital Bellevue, Neurologie, Saint-Etienne, France
  6. 6
    Hôpital de la Pitié-Salpêtrière, Fédération Mazarin, Paris, France
  7. 7
    Hospices Civils de Lyon, Pôle IMER, Lyon, France
  8. 8
    The Ohio State University, Department of Psychiatry, Columbus, Ohio, USA
  9. 9
    Service of Neurology, Hospital Clinic, Universitat de Barcelona and Institut d’Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Barcelona, Spain
  1. Dr J Honnorat, Neurologie B, Hôpital Neurologique de Lyon, 59 boulevard Pinel, 69677 Bron Cedex, France; jerome.honnorat{at}chu-lyon.fr

Abstract

Objective: Anti-Hu antibodies (Hu-Ab) and anti-CV2/CRMP5 antibodies (CV2/CRMP5-Ab) have been identified in association with paraneoplastic neurological disorders. However, it is not clear whether these antibodies are associated with specific neurological symptoms or are only markers of anti-cancer immune reaction.

Methods: To address this question, 37 patients with CV2/CRMP5-Ab and 324 patients with Hu-Ab were compared.

Results: Whereas the age and sex ratio were the same between the two groups, the distribution of neurological symptoms was not. Patients with CV2/CRMP5-Ab presented more frequently cerebellar ataxia, chorea, uveo/retinal symptoms and myasthenic syndrome (Lambert–Eaton myasthenic syndrome LEMS or myasthenia gravis). They also had a better Rankin score. In contrast, dysautonomia, brainstem encephalitis and peripheral neuropathy were more frequent in patients with Hu-Ab. Limbic encephalitis occurred similarly in both groups. Small-cell lung cancer was the most frequently associated tumour in both groups of patients, while malignant thymoma was observed only in patients with CV2/CRMP5-Ab. In particular, patients with CV2/CRMP5-Ab and thymoma developed myasthenic syndrome more frequently, while patients with SCLC developed neuropathies more frequently. Chorea and myasthenic syndrome were only seen in patients with CV2/CRMP5-Ab. The median survival time was significantly longer in patients with CV2/CRMP5-Ab, and this effect was not dependent on the type of tumour.

Interpretation: The data demonstrate that in patients with paraneoplastic neurological syndromes, the neurological symptoms and survival vary with both the type of associated onco-neural antibody and the type of tumour.

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Footnotes

  • Competing interests: None.

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