Onco-neural antibodies and tumour type determine survival and neurological symptoms in paraneoplastic neurological syndromes with Hu or CV2/CRMP5 antibodies
- J Honnorat1,2,3,
- S Cartalat-Carel1,
- D Ricard4,
- J Ph Camdessanche1,2,5,
- A F Carpentier1,6,
- V Rogemond1,2,
- F Chapuis3,7,
- M Aguera2,
- E Decullier3,7,
- A M Duchemin8,
- F Graus9,
- J C Antoine1,2,4
- 1Centre de Référence Maladies Rares “Syndromes Neurologiques Paranéoplasiques,” Hospices Civils de Lyon, Hôpital Neurologique, Bron, France
- 2UMR INSERM S842, Lyon, France
- 3Université de Lyon, Université Lyon 1, Lyon, France
- 4Hôpital du Val de Grâce, Neurologie, Paris, France
- 5Hôpital Bellevue, Neurologie, Saint-Etienne, France
- 6Hôpital de la Pitié-Salpêtrière, Fédération Mazarin, Paris, France
- 7Hospices Civils de Lyon, Pôle IMER, Lyon, France
- 8The Ohio State University, Department of Psychiatry, Columbus, Ohio, USA
- 9Service of Neurology, Hospital Clinic, Universitat de Barcelona and Institut d’Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Barcelona, Spain
- Dr J Honnorat, Neurologie B, Hôpital Neurologique de Lyon, 59 boulevard Pinel, 69677 Bron Cedex, France; jerome.honnorat{at}chu-lyon.fr
- Received 24 October 2007
- Revised 25 June 2008
- Accepted 8 August 2008
- Published Online First 17 October 2008
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.
Footnotes
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Competing interests: None.
