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Malignant tumors in autoimmune encephalitis with anti-NMDA receptor antibodies

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Abstract

Objective

The aim of this study was to describe specificities of patients with NMDA receptor antibody (NMDAR-Ab) encephalitis associated with a malignant tumor.

Methods

Retrospective observational study of 252 patients with NMDAR-Ab encephalitis of the French Paraneoplastic Neurological Syndrome Reference Center. Patients were classified in three groups: (1) non-malignant ovarian teratomas, (2) malignant ovarian teratomas (immature), and (3) other malignant tumors.

Results

Sixty patients (23.8%) had an associated tumor and 15 (6%) were malignant. No particular neurological symptom was observed in these patients. Ovarian teratomas were the most frequent (51 cases) with 6 of them immature (11.8% of teratomas). Nine patients (3.6%) developed other malignant tumors (3 small cell lung carcinomas, 1 uterine adenocarcinoma, 1 prostate adenocarcinoma, 1 Hodgkin lymphoma, 1 pineal dysgerminoma, 1 neuroblastoma and 1 pancreatic neuroendocrine tumor). Among patients with a cancer other than teratoma, 6/9 were elderly patients (median age 65 years, representing 30% of elderly patients with such encephalitis) compared to a median age of 26 years in adult patients included herein. The clinical course was similar in the three groups, other than a higher death rate among patients with malignant tumors (86 versus 2%; p < 0.001) mainly due to tumor progression (5/7 deaths).

Conclusion

Immature ovarian teratomas represent 11.8% of all teratomas in patients with NDMAR-Ab encephalitis. The other malignant tumors are mainly observed in elderly patients. The presence of a malignant tumor does not impact the neurological presentation but is directly associated with a higher risk of death.

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Acknowledgements

We gratefully thank Dr Lara Chalabreysse and Dr Isabelle Treilleux for the helpful collaboration. This study was supported by research Grants from Agence Nationale de la Recherche (ANR-14-CE15-0001-MECANO), the Fondation pour la recherche sur le cerveau (FRC-Neurodon2014) and CSL Behring France, FRM (Fondation pour la recherche médicale) DQ20170336751.

Author information

Author notes

  1. Chloé Bost and Eve Chanson contributed equally to this manuscript.

Authors and Affiliations

  1. French Reference Center for Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, 69677, Bron, France

    Chloé Bost, Eve Chanson, Géraldine Picard, François Ducray, Veronique Rogemond, Dimitri Psimaras, Jean-Christophe Antoine, Jean-Yves Delattre, Virginie Desestret & Jerome Honnorat

  2. Institut NeuroMyoGene INSERM U1217/CNRS UMR 5310, Université de Lyon, Université Claude Bernard Lyon 1, 69372, Lyon, France

    Chloé Bost, Eve Chanson, Marie-Eve Mayeur, François Ducray, Veronique Rogemond, Jean-Christophe Antoine, Virginie Desestret & Jerome Honnorat

  3. University of Lyon, Université Claude Bernard Lyon 1, Lyon, 69372, Lyon, France

    Chloé Bost, Eve Chanson, David Meyronet, François Ducray, Virginie Desestret & Jerome Honnorat

  4. INSERM 1052, CNRS 5286, Centre Leon Berard, Centre de Recherche en Cancérologie de Lyon, 69008, Lyon, France

    David Meyronet & François Ducray

  5. Groupe Hospitalier Pitié-Salpêtrière, Service de neurologie 2-Mazarin, AP-HP, 75013, Paris, France

    Dimitri Psimaras & Jean-Yves Delattre

  6. Service de Neurologie, CHU de Saint-Etienne et Université de Lyon, 42023, Saint-Etienne, France

    Jean-Christophe Antoine

  7. Inserm, U 1127, CNRS, UMR 7225, ICM, Sorbonne Universités, UPMC Univ. Paris 06, UM 75 Paris, 75013, Paris, France

    Jean-Yves Delattre

  8. Neuro-Oncologie, Hôpital Neurologique Pierre Wertheimer, 59 Boulevard Pinel, 69677, Bron Cedex, France

    Jerome Honnorat

Authors
  1. Chloé Bost
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  2. Eve Chanson
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  3. Géraldine Picard
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  11. Virginie Desestret
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Contributions

Dr Chloé Bost: analysis, interpretation of the data, drafting the manuscript for intellectual content. Dr Eve Chanson: analysis, interpretation of the data, drafting the manuscript for intellectual content. Ms Géraldine Picard: acquisition and analysis of data. Dr David Meyronet: acquisition and analysis of data. Ms Marie-Eve Mayeur: acquisition and analysis of data. Dr François Ducray: acquisition of data, critical revision of the manuscript for important intellectual content. Dr Véronique Rogemond: acquisition and analysis of data. Dr Dimitri Psimaras: acquisition of data, critical revision of the manuscript for important intellectual content. Pr Jean-Christophe Antoine: acquisition of data, critical revision of the manuscript for important intellectual content. Pr Jean-Yves Delattre: critical revision of the manuscript for important intellectual content. Dr Virginie Desestret: analysis and interpretation, critical revision of the manuscript for important intellectual content, study supervision. Pr Jérôme Honnorat: study concept and design, analysis and interpretation, critical revision of the manuscript for important intellectual content, study supervision.

Corresponding author

Correspondence to Jerome Honnorat.

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Conflicts of interest

This study is not industry-sponsored. Dr Chloé Bost reports no disclosures; Dr Eve Chanson reports no disclosures; Ms Géraldine Picard reports no disclosures; Dr David Meyronet reports no disclosures; Ms Marie-Eve Mayeur reports no disclosures; Dr François Ducray reports no disclosures; Dr Véronique Rogemond reports no disclosures; Dr Dimitri Psimaras and Pr Jean-Christophe Antoine report no disclosures; Pr Jean-Yves Delattre reports no disclosures; Dr Virginie Desestret reports no disclosures; Pr Jérôme Honnorat reports no disclosures.

Ethical standards

Written consent was obtained from all patients, and this study was approved by the institutional review board of the University Claude Bernard Lyon 1 and Hospices Civils de Lyon.

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Bost, C., Chanson, E., Picard, G. et al. Malignant tumors in autoimmune encephalitis with anti-NMDA receptor antibodies. J Neurol 265, 2190–2200 (2018). https://doi.org/10.1007/s00415-018-8970-0

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  • DOI: https://doi.org/10.1007/s00415-018-8970-0

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