Seronegative paraneoplastic cerebellar degeneration: the PNS Euronetwork experience

Authors

  • F. Ducray,

    1. French Reference Center on Paraneoplastic Neurological Syndrome, Department of Neuro-oncology, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France
    2. Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292, Lyon, France
    3. Université de Lyon – Université Claude Bernard Lyon 1, Lyon, France
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    • These authors contributed equally.
  • G. Demarquay,

    1. French Reference Center on Paraneoplastic Neurological Syndrome, Department of Neuro-oncology, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France
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    • These authors contributed equally.
  • F. Graus,

    1. Service of Neurology, Hospital Clínic, Universitat de Barcelona and Institut d′Investigació Biomèdica August Pi i Suyer (IDIBAPS), Barcelona, Spain
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  • E. Decullier,

    1. Université de Lyon – Université Claude Bernard Lyon 1, Lyon, France
    2. Hospices Civils de Lyon, Pôle Information Médicale Evaluation Recherche, Unité de Recherche Clinique, Lyon, France
    3. Université de Lyon, RECIF, Lyon, France
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  • J.-C. Antoine,

    1. French Reference Center on Paraneoplastic Neurological Syndrome, Department of Neuro-oncology, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France
    2. Hôpital Bellevue, Neurologie, Saint-Etienne, France
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  • B. Giometto,

    1. Department of Neurology, Ospedale Ca’ Foncello, Treviso, Italy
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  • D. Psimaras,

    1. French Reference Center on Paraneoplastic Neurological Syndrome, Department of Neuro-oncology, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France
    2. Service de Neurologie 2 – Division Mazarin, Groupe Hospitalier Pitié-Salpêtrière, APHP, Paris, France
    3. Université Pierre et Marie Curie − Paris 6, Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière, UMR, Faculté de Médecine site Pitié-Salpêtrière, Paris, France
    4. INSERM, Paris, France
    5. CNRS, UMR, Paris, France
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  • J.-Y. Delattre,

    1. French Reference Center on Paraneoplastic Neurological Syndrome, Department of Neuro-oncology, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France
    2. Service de Neurologie 2 – Division Mazarin, Groupe Hospitalier Pitié-Salpêtrière, APHP, Paris, France
    3. Université Pierre et Marie Curie − Paris 6, Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière, UMR, Faculté de Médecine site Pitié-Salpêtrière, Paris, France
    4. INSERM, Paris, France
    5. CNRS, UMR, Paris, France
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  • A. F. Carpentier,

    1. Université Paris 13, UFR de Santé, Médecine et Biologie Humaine, Bobigny, France
    2. Assistance Publique − Hôpitaux de Paris (AP-HP), Hôpital Avicenne, Service de Neurologie, Bobigny, France
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  • J. Honnorat

    Corresponding author
    1. French Reference Center on Paraneoplastic Neurological Syndrome, Department of Neuro-oncology, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France
    2. Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292, Lyon, France
    3. Université de Lyon – Université Claude Bernard Lyon 1, Lyon, France
    • Correspondence: J. Honnorat, Neuro-oncologie, Hôpital Neurologique Pierre Wertheimer, 59 boulevard Pinel, 69677 Bron Cedex, France (tel.: +04 72 35 78 08; fax: 04 72 35 73 29; e-mail: jerome.honnorat@chu-lyon.fr).

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Abstract

Background and purpose

To describe the characteristics of patients presenting a paraneoplastic cerebellar degeneration without classical onconeural antibodies (seronegative PCD).

Methods

Thirty-nine seronegative PCD patients from the Paraneoplastic Neurological Syndrome Euronetwork were retrospectively analyzed and compared with 180 patients with PCD associated with classical onconeural antibodies (seropositive PCD).

Results

No patient had anti-CASPR2 or anti-mGluR1 antibodies. No significant difference between the clinical characteristics of seronegative and seropositive PCD patients was observed. Yet the frequency of associated tumors was different. Lymphoma was more frequent in seronegative than in seropositive women (24% vs. 2%, P = 0.002) whilst gynecological cancer were less frequent (38% vs. 74%, P = 0.002). In comparison with seropositive men, seronegative men more frequently had a non-small-cell lung cancer (27% vs. 6%, P = 0.08) or a genitourinary cancer (22% vs. 0%, P = 0.04) but less frequently a small-cell lung cancer (23% vs. 74%, P = 0.002). Seronegative and seropositive PCD patients with similar tumors had a similar overall survival.

Conclusion

The clinical characteristics of seronegative and seropositive PCD are similar but the spectrum of associated tumors is different. The immunological scenario of seronegative PCD seems to be different from that of limbic encephalitis with only few patients harboring anti-neuropile antibodies.

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