Seronegative paraneoplastic cerebellar degeneration: the PNS Euronetwork experience

被引:36
作者
Ducray, F. [1 ,2 ,3 ]
Demarquay, G. [1 ]
Graus, F. [4 ,5 ]
Decullier, E. [3 ,6 ,7 ]
Antoine, J. -C. [1 ,8 ]
Giometto, B. [9 ]
Psimaras, D. [1 ,10 ,11 ,12 ,13 ]
Delattre, J. -Y. [1 ,10 ,11 ,12 ,13 ]
Carpentier, A. F. [14 ,15 ]
Honnorat, J. [1 ,2 ,3 ]
机构
[1] Hop Neurol, Hosp Civils Lyon, Dept Neurooncol, French Reference Ctr Paraneoplast Neurol Syndrome, Bron, France
[2] Lyon Neurosci Res Ctr, INSERM, U1028, CNRS,UMR 5292, Lyon, France
[3] Univ Lyon 1, F-69365 Lyon, France
[4] Univ Barcelona, Hosp Clin, Serv Neurol, Barcelona, Spain
[5] Inst Invest Biomed August Pi & Suyer IDIBAPS, Barcelona, Spain
[6] Hosp Civils Lyon, Unite Rech Clin, Lyon, France
[7] Univ Lyon, RECIF, EA Sante Individu Soc 4129, Lyon, France
[8] Hop Bellevue, St Etienne, France
[9] Osped Ca Foncello, Dept Neurol, Treviso, Italy
[10] Grp Hosp Pitie Salpetriere, APHP, Div Mazarin, Serv Neurol 2, F-75634 Paris, France
[11] Univ Paris 06, Ctr Rech, Inst Cerveau & Moelle Epiniere, UMR,Fac Med,Site Pitie Salpetriere, Paris, France
[12] INSERM, Paris, France
[13] CNRS, UMR, Paris, France
[14] Univ Paris 13, UFR Sante Med & Biol Humaine, Bobigny, France
[15] Hop Avicenne, AP HP, Serv Neurol, F-93009 Bobigny, France
关键词
onconeural antibodies; paraneoplastic cerebellar degeneration; LUNG-CANCER; ANTIBODIES;
D O I
10.1111/ene.12368
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Background and purposeTo describe the characteristics of patients presenting a paraneoplastic cerebellar degeneration without classical onconeural antibodies (seronegative PCD). MethodsThirty-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). ResultsNo 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. ConclusionThe 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.
引用
收藏
页码:731 / 735
页数:5
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