Chloé Bost1,2,3, Eve Chanson1,2,3, Géraldine Picard1, David Meyronet3,4, Marie-Eve Mayeur2, François Ducray1,2,3,4, Veronique Rogemond1,2, Dimitri Psimaras1,5, Jean-Christophe Antoine1,2,6, Jean-Yves Delattre1,5,7, Virginie Desestret1,2,3, Jerome Honnorat8,9,10,11. 1. French Reference Center for Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, 69677, Bron, France. 2. Institut NeuroMyoGene INSERM U1217/CNRS UMR 5310, Université de Lyon, Université Claude Bernard Lyon 1, 69372, Lyon, France. 3. University of Lyon, Université Claude Bernard Lyon 1, Lyon, 69372, Lyon, France. 4. INSERM 1052, CNRS 5286, Centre Leon Berard, Centre de Recherche en Cancérologie de Lyon, 69008, Lyon, France. 5. Groupe Hospitalier Pitié-Salpêtrière, Service de neurologie 2-Mazarin, AP-HP, 75013, Paris, France. 6. Service de Neurologie, CHU de Saint-Etienne et Université de Lyon, 42023, Saint-Etienne, France. 7. Inserm, U 1127, CNRS, UMR 7225, ICM, Sorbonne Universités, UPMC Univ. Paris 06, UM 75 Paris, 75013, Paris, France. 8. French Reference Center for Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, 69677, Bron, France. jerome.honnorat@chu-lyon.fr. 9. Institut NeuroMyoGene INSERM U1217/CNRS UMR 5310, Université de Lyon, Université Claude Bernard Lyon 1, 69372, Lyon, France. jerome.honnorat@chu-lyon.fr. 10. University of Lyon, Université Claude Bernard Lyon 1, Lyon, 69372, Lyon, France. jerome.honnorat@chu-lyon.fr. 11. Neuro-Oncologie, Hôpital Neurologique Pierre Wertheimer, 59 Boulevard Pinel, 69677, Bron Cedex, France. jerome.honnorat@chu-lyon.fr.
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.
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-Abencephalitis. 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.
Authors: U Wagner; P Harter; F Hilpert; S Mahner; A Reuß; A du Bois; E Petru; W Meier; P Ortner; K König; K Lindel; D Grab; P Piso; O Ortmann; I Runnebaum; J Pfisterer; D Lüftner; N Frickhofen; F Grünwald; B O Maier; J Diebold; S Hauptmann; F Kommoss; G Emons; B Radeleff; M Gebhardt; N Arnold; G Calaminus; I Weisse; J Weis; J Sehouli; D Fink; A Burges; A Hasenburg; C Eggert Journal: Geburtshilfe Frauenheilkd Date: 2013-09 Impact factor: 2.915
Authors: Josep Dalmau; Amy J Gleichman; Ethan G Hughes; Jeffrey E Rossi; Xiaoyu Peng; Meizan Lai; Scott K Dessain; Myrna R Rosenfeld; Rita Balice-Gordon; David R Lynch Journal: Lancet Neurol Date: 2008-10-11 Impact factor: 44.182
Authors: F Graus; J Y Delattre; J C Antoine; J Dalmau; B Giometto; W Grisold; J Honnorat; P Sillevis Smitt; Ch Vedeler; J J G M Verschuuren; A Vincent; R Voltz Journal: J Neurol Neurosurg Psychiatry Date: 2004-08 Impact factor: 10.154
Authors: Marcus Dabner; W Glenn McCluggage; Chris Bundell; Aisling Carr; Yee Leung; Raghwa Sharma; Colin J R Stewart Journal: Int J Gynecol Pathol Date: 2012-09 Impact factor: 2.762
Authors: Marc Nikolaus; Arend Koch; Werner Stenzel; Sefer Elezkurtaj; Felix Sahm; Anna Tietze; Laura Stöffler; Jakob Kreye; Pablo Hernáiz Driever; Ulrich W Thomale; Angela M Kaindl; Markus Schuelke; Ellen Knierim Journal: Acta Neuropathol Date: 2022-06-21 Impact factor: 15.887
Authors: Karol Perlejewski; Agnieszka Pawełczyk; Iwona Bukowska-Ośko; Małgorzata Rydzanicz; Tomasz Dzieciątkowski; Marcin Paciorek; Michał Makowiecki; Kamila Caraballo Cortés; Marta Grochowska; Marek Radkowski; Tomasz Laskus Journal: Open Forum Infect Dis Date: 2020-10-07 Impact factor: 3.835
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
Authors: Chae Hyeong Lee; Eun Jung Kim; Min Hee Lee; Ga Won Yim; Kyeong Joon Kim; Kwang Ki Kim; Eo Jin Kim; Ju Won Roh Journal: J Korean Med Sci Date: 2020-06-22 Impact factor: 2.153