F Ducray1, G Demarquay, F Graus, E Decullier, J-C Antoine, B Giometto, D Psimaras, J-Y Delattre, A F Carpentier, J Honnorat. 1. French Reference Center on Paraneoplastic Neurological Syndrome, Department of Neuro-oncology, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292, Lyon, France; Université de Lyon - Université Claude Bernard Lyon 1, Lyon, France.
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.
BACKGROUND AND PURPOSE: To describe the characteristics of patients presenting a paraneoplastic cerebellar degeneration without classical onconeural antibodies (seronegative PCD). METHODS: Thirty-nine seronegative PCDpatients 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 PCDpatients 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 PCDpatients 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.
Authors: Nicolas De Leiris; Berangère Ruel; Jean Vervandier; José Boucraut; Stephan Grimaldi; Tatiana Horowitz; Jean Pelletier; Frederique Fluchere; Jacques-Yves Campion; Elsa Kaphan; Eric Guedj Journal: Eur J Nucl Med Mol Imaging Date: 2021-08-31 Impact factor: 9.236
Authors: Francesc Graus; Alberto Vogrig; Sergio Muñiz-Castrillo; Jean-Christophe G Antoine; Virginie Desestret; Divyanshu Dubey; Bruno Giometto; Sarosh R Irani; Bastien Joubert; Frank Leypoldt; Andrew McKeon; Harald Prüss; Dimitri Psimaras; Laure Thomas; Maarten J Titulaer; Christian A Vedeler; Jan J Verschuuren; Josep Dalmau; Jerome Honnorat Journal: Neurol Neuroimmunol Neuroinflamm Date: 2021-05-18