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380 results on '"Delmont, Emilien"'

1. Clinical, paraclinical and outcome features of 166 patients with acute anti-GQ1b antibody syndrome

Author

Coly, Martin, Adams, David, Attarian, Shahram, Bouhour, Françoise, Camdessanché, Jean-Philippe, Carey, Guillaume, Cauquil, Cécile, Chanson, Jean-Baptiste, Chrétien, Pascale, Créange, Alain, Delmont, Emilien, Fargeot, Guillaume, Frachet, Simon, Gendre, Thierry, Kuntzer, Thierry, Labeyrie, Céline, Maisonobe, Thierry, Michaud, Maud, Moulin, Maximilien, Nicolas, Guillaume, Noury, Jean-Baptiste, Péréon, Yann, Puma, Angela, Sole, Guilhem, Taithe, Frédéric, Tard, Céline, Théaudin, Marie, Timsit, Serge, Venditti, Laura, and Echaniz-Laguna, Andoni

Published
2024
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6. Functional and Molecular Characterization of New SPTLC1 Missense Variants in Patients with Hereditary Sensory and Autonomic Neuropathy Type 1 (HSAN1)

Author

Rochat, Julie, primary, Blavier, André, additional, Ruet, Séverine, additional, Vasseur, Sophie, additional, Puma, Angela, additional, Desnous, Béatrice, additional, Chan, Victor, additional, Delmont, Emilien, additional, Attarian, Shahram, additional, Juntas Morales, Raul, additional, Quadrio, Isabelle, additional, Vidoni, Léo, additional, Bonello-Palot, Nathalie, additional, and Cheillan, David, additional

Published
2024
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8. Profils clinique, électrophysiologique et biologique des neuropathies anti-MAG en fonction du statut mutationnel MYD88 et de l’hémopathie sous-jacente

Author

Guérémy, Alexandre, primary, Delmont, Emilien, additional, Attarian, Shahram, additional, Grapperon, Aude-Marie, additional, Kouton, Ludivine, additional, Farnault, Laure, additional, Boudjarane, John, additional, Gabert, Jean, additional, Fortanier, Etienne, additional, and Boucraut, José, additional

Published
2024
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15. Molecular Imaging in CANVAS: A Contribution for Differential Diagnosis?

Author

Horowitz, Tatiana, Guedj, Eric, Eusebio, Alexandre, Fluchère, Frédérique, Azulay, Jean‐Philippe, Delmont, Emilien, and Grimaldi, Stephan

Subjects
MULTIPLE system atrophy, CEREBELLUM degeneration, SINGLE-photon emission computed tomography, POSITRON emission tomography, DIFFERENTIAL diagnosis
Abstract

Background: Phenotypes of CANVAS are increasingly diversified, including bradykinesia and dysautonomia, so that its primary differential diagnoses are multiple system atrophy‐cerebellar type (MSA‐c), and spinocerebellar ataxia type 3 (SCA3). This case series aims to highlight key molecular imaging findings in CANVAS. Cases: We report a case series of six patients with CANVAS who underwent nuclear medicine examinations in our center and 13 patients from the literature. These include 18F‐FDG brain positron emission tomography (PET), single photon emission computed tomography (SPECT) of dopamine transporter (DaT) activity, and 123I‐MIBG cardiac scintigraphy of noradrenergic transmission. Conclusions: In CANVAS, 18F‐FDG brain PET mainly shows cerebellar hypometabolism, with preserved brainstem and striatum metabolism, contrasting with SCA3 and MSA‐c. Dopaminergic denervation on scintigraphy seems to be associated with clinical parkinsonism, ranging from normal to severely impaired DaT SPECT. Additionally, 123I‐MIBG cardiac scintigraphy might show denervation in CANVAS, similar to SCA3, but not in most MSA‐c patients. [ABSTRACT FROM AUTHOR]

Published
2024
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16. Longitudinal follow‐up and prognostic factors in nitrous oxide‐induced neuropathy.

Author

Fortanier, Etienne, Delmont, Emilien, Corazza, Giovanni, Kouton, Ludivine, Micallef, Joelle, and Attarian, Shahram

Subjects
*RISK assessment, *NITROUS oxide, *DESCRIPTIVE statistics, *RETROSPECTIVE studies, *NEUROLOGICAL disorders, *LONGITUDINAL method, *ELECTROPHYSIOLOGY
Abstract

Background and Aim: Recreational use of nitrous oxide (N2O) has been associated with the development of severe nitrous oxide‐induced neuropathy (N2On). Follow‐up of these patients poses challenges, and their clinical progression remains largely unknown. The identification of prognostic factors is made difficult by the lack of standardized longitudinal assessments in most studies. The objective was to document the course of neuropathy through systematic follow‐up assessments in N2On patients to identify prognostic factors for persistent disability after 6 months. Methods: We gathered demographic, clinical, biological, and electrophysiological data from N2On patients hospitalized in the Referral center in Marseille, both at baseline and during a standardized follow‐up assessment at 6 months. Results: We retrospectively included 26 N2On patients (mean age 22.6 ± 4.4). Significant improvements were observed in all main clinical scores including Rankin, ONLS, and MRC testing (p <.01). Electrophysiological studies (EDX) revealed a predominantly motor neuropathy with marked reduction in CMAP in the lower limbs at baseline, and no significant improvement in motor parameters (p =.543). Rankin score at 6 months correlated with the initial weekly N2O consumption (r =.43, p =.03) and the CMAP sum score in the lower limbs at the first EDX (r = −.47, p =.02). Patients with and without myelitis showed similar Rankin and ONLS score after 6 months. Interpretation: The clinical course generally improved favorably at 6 months with notable amelioration in the primary disability scores, sensory deficits, and ataxia. However, distal motor impairment associated with peripheral neuropathy persisted, with distal axonal loss emerging as the main prognostic factor for long‐term disability in these young patients. [ABSTRACT FROM AUTHOR]

Published
2024
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17. Contactin-1 is a novel target antigen in membranous nephropathy associated with chronic inflammatory demyelinating polyneuropathy

Author

Le Quintrec, Moglie, Teisseyre, Maxime, Bec, Nicole, Delmont, Emilien, Szwarc, Ilan, Perrochia, Hélène, Machet, Marie Christine, Chauvin, Anthony, Mavroudakis, Nicolas, Taieb, Guillaume, Lanfranco, Luca, Rigothier, Claire, José, Boucraut, Concetta, Catalano, Geneste, Clair, Pernin, Vincent, Larroque, Christian, Devaux, Jérôme, and Beyze, Anaïs

Published
2021
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19. Safety, efficacy, and tolerability of efgartigimod in patients with generalised myasthenia gravis (ADAPT): a multicentre, randomised, placebo-controlled, phase 3 trial

Author

De Bleecker, Jan L., De Koning, Kathy, De Mey, Katrien, De Pue, Annelien, Mercelis, Rudolf, Wyckmans, Maren, Vinck, Caroline, Wagemaekers, Linda, Baets, Jonathan, Ng, Eduardo, Shabanpour, Jafar, Daniyal, Lubna, Mannan, Shabber, Katzberg, Hans D., Genge, Angela, Siddiqi, Zaeem, Junkerová, Jana, Horakova, Jana, Reguliova, Katerina, Tyblova, Michaela, Jurajdova, Ivana, Novakova, Iveta, Jakubikova, Michala, Pitha, Jiri, Vohanka, Stanislav, Havelkova, Katerina, Horak, Tomas, Bednarik, Josef, Horakova, Mageda, Meisel, Andreas, Remstedt, Dike, Heibutzki, Claudia, Kohler, Siegfried, Gerischer, Lea, Hoffman, Sarah, Stascheit, Frauke, Vissing, John, Zafirakos, Lizzie, Khatri, Kuldeep Kumar, Autzen, Anne, Godtfeldt Stemmerik, Mads Peter, Andersen, Henning, Attarian, Shahram, Salort-Campana, Emmanuelle, Delmont, Emilien, Grapperon, Aude-Marie, Kouton, Ludivine, Tsiskaridze, Alexander, Rózsa, Csilla, Jakab, Gedeonne Margo, Toth, Szilvia, Szabo, Gyorgyi, Bors, David, Szabo, Eniko, Campanella, Angela, Vanoli, Fiammetta, Frangiamore, Rita, Antozzi, Carlo, Bonanno, Silvia, Maggi, Lorenzo, Giossi, Riccardo, Saccà, Francesco, Marsili, Angela, Pane, Chiara, Puorro, Giorgia, Reia, Antonio, Antonini, Giovanni, Alfieri, Girolamo, Morino, Stefania, Garibaldi, Matteo, Fionda, Laura, Leonardi, Luca, Konno, Shingo, Uzawa, Akiyuki, Sakuma, Kaoru, Watanabe, Chiho, Ozawa, Yukiko, Yasuda, Manato, Onishi, Yosuke, Samukawa, Makoto, Tsuda, Tomoko, Suzuki, Yasushi, Ishida, Sayaka, Watanabe, Genya, Takahashi, Masanori, Nakamura, Hiroko, Sugano, Erina, Kubota, Tomoya, Imai, Tomihiro, Suzuki., Mari, Mori, Ayako, Yamamoto, Daisuke, Ikeda, Kazuna, Hisahara, Shin, Masuda, Masayuki, Takaki, Miki, Minemoto, Kanako, Ido, Nobuhiro, Naito, Makiko, Okubo, Yoshihiko, Sugimoto, Takamichi, Takematsu, Yuka, Kamei, Ayumi, Shimizu, Mihiro, Naito, Hiroyuki, Nomura, Eiichi, Van Heur, Marjolein, Peters, Anne-Marie, Tannemaat, Martijn, Ruiter, Annabel, Keene, Kevin, Halas, Marek, Szczudlik, Andrzej, Pinkosz, Marta, Frasinska, Monika, Zwolinska, Grazyna, Kostera-Pruszczyk, Anna, Golenia, Aleksandra, Szczudlik, Piotr, Szczechowski, Lech, Pasko, Aneta, Poverennova, Irina, Urtaeva, Lubov, Kuznetsova, Nadezhda, Romanova, Tatiana, Nadezhda, Malkova, Lapochka, Elena, Korobko, Denis, Vergunova, Ilona, Melnikova, Anna, Bulatova, Ekaterina, Antipenko, Elena, Basta, Ivana, Bozovic, Ivo, Lavrnic, Dragana, Stojanovic, Vidosava Rakocevic, Beydoun, Said, Akhter, Salma, Malekniazi, Ali, Darki, Leila, Pimentel, Norianne, Cannon, Victoria, Chopra, Manisha, Traub, Rebecca, Mozaffar, Tahseen, Hernandez, Isela, Turner, Ivonne, Habib, Ali, Goyal, Namita, Kak, Manisha, Velasquez, Erik, Lam, Lucy, Suresh, Niraja, Farias, Jerrica, Jones, Sarah, Wagoner, Mary, Eggleston, Debbie, Bertorini, Tulio, Benzel, Cindy, Henegar, Robert, Pillai, Rekha, Bharavaju-Sanka, Ratna, Paiz, Carolyn, Jackson, Carlayne, Ruzhansky, Katherine, Dimitrova, Diana, Visser, Amy, Chahin, Nizar, Levine, Todd, Lisak, Robert, Jia, Kelly, Mada, Flicia, Bernitsas, Evanthia, Pasnoor, Mamatha, Roath, Katherine, Colgan, Samantha, Currence, Melissa, Heim, Andrew, Barohn, Richard, Dimachkie, Mazen, Statland, Jeffrey, Jawdat, Omar, Jabari, Duaa, Farmakidis, Constantine, Gilchrist, James, Li, Yuebing, Caristo, Irys, Hastings, Debbie, Morren, John Anthony, Weiss, Michael, Muppidi, Srikanth, Nguyen, Tia, Welsh, Lesly, So, Yuen, Goyal, Neelam, Pulley, Michael, Bailey, Cathy, Quraishi, Zubair, Berger, Alan, Sahagian, Gregory, Camberos, Yasmin, Frishberg, Benjamin, Howard, James F, Jr, Bril, Vera, Vu, Tuan, Karam, Chafic, Peric, Stojan, Margania, Temur, Murai, Hiroyuki, Bilinska, Malgorzata, Shakarishvili, Roman, Smilowski, Marek, Guglietta, Antonio, Ulrichts, Peter, Vangeneugden, Tony, Utsugisawa, Kimiaki, Verschuuren, Jan, and Mantegazza, Renato

Published
2021
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20. Long-term safety and efficacy of patisiran for hereditary transthyretin-mediated amyloidosis with polyneuropathy: 12-month results of an open-label extension study

Author

Vita, Giuseppe, Rizzo, Vincenzo, Russo, Massimo, Mazzeo, Anna, Gentile, Luca, Berk, John L, Brueckner, Caitlin, Lazzari, Victoria, Wiesman, Janice, DeLong, Douglas, Victory, Jennifer, Dalton, James, May, John, Gilmore, Catherine, Attarian, Shahram, Diallo, Saran, Delmont, Emilien, Pouget, Jean, Verschueren, Annie, Grapperon, Aude-Marie, Campana-Salort, Emmanuelle, Conceição, Isabel M, Lopes, Ana, Lamas, Filipa, Neves, Carlos, Castro, Jose, Pereira, Pedro, Castro, Isabel, Franco, Ana, Santos, Miguel Oliveira, de Azevedo Coutinho, Conceição, Falcao de Campos, Catarina, Coelho, Teresa, Hipólito Reis, Antonio, Correia, Nuno, Perez, Javier M, Martins da Silva, Ana, Alves, Cristina, Cardoso, Marcio, Valdrez, Katia, Monte, Julia R, Pessoa, Bernardete, Guimaraes, Nadia, Freitas, Monica, Ramalho, Joana, Ferreira, Natalia, Kuzume, Daisuke, Tard, Celine, Waucquier, Nawal, Rougeaux, Isabelle, Brice, Sylvie, Kasprzyk, Emmanuelle, Elrezzi, Elise, Meguig, Sayah, Hachulla, Eric, Gauvain, Clement, Migaud-Chervy, Maria-Claire, Deplanque, Dominique, Jozefowicz, Elsa, Lebellec, Loic, Adams, David, Balaya-Gouraya, Line, Jehan Lacour, Nathalie, Bournane, Halima, Martin, Nathalie, Elabed, Mongia, Sacko, Niamey, Boubrit, Yasmine, Gaouar, Amina, Rakotondratafika, Fetra, Théaudin-Saliou, Marie, Cauquil-Michon, Cécile, Labeyrie, Celine, Not, Adeline, Al-Salameh, Abdallah, Lecoq, Anne-Lise, Stephant, Maeva, Echaniz-Laguna, Andoni, Becquemont, Laurent, Beaudonnet, Guillemette, Algalarrondo, Vincent, Eliahou, Ludivine, Slama, Michel S, Rousseau, Antoine, Signate, Aissatou, Berthelot, Emeline, Inamo, Jocelyn, Planté-Bordeneuve, Violaine, Vervoitte, Laetitia, Focseneanu, Cecile, Gendre, Thierry, Arrouasse, Raphaele, Ayache, Samar S., Ernande, Laura, Le Corvoisier, Philippe, Salhi, Hayet, Choumert, Ariane, Ehinger, Vincent, Ruiz, Julie, Charlin, Cyril, Megelin, Thomas, Brannagan III, Thomas H, Fayerman, Raisy, Kim, Arreum, Paras, Allan, Gonzalez, Leidy J, Tsang, Steven, Wajnsztajn, Fernanda, Shije, Jeffrey, Ulane, Christina, Kleyman, Inna, Weimer, Louis, Cioroiu, Comana, Lambrianides, Sakis, Abu-Manneh, Rana, Zamba-Papanicolaou, Eleni, Agathangelou, Petros, Leonidou, Eleni, Tada, Satoshi, Fujita, Akemi, Nagai, Masahiro, Ando, Rina, Hosokawa, Yuko, Yamanishi, Yuki, Overcash, J. Scott, Giardino, Elena, Boyer, Leslie, Dang, Lien, Le, An, Nguyen, Tyler, Giang, Lien, Sellers, Peter, Tran, Leyla, Truong, Nghi, Vinas, Maita, Hrkman, Nicole, Miller, Sarah, Nguyen, David, Smith, Ashley, Pu, Helen, Li, Steve, Vuong, Thao, Dioso, Holly, Green, Sinikka, Lee, Kia, Chu, Hanh, Waters, Michael, Coskun, Derya J, Zepeda, Karla A, O'Riordan, William, Obici, Laura, Cortese, Andrea, Lozza, Alessandro, Merlini, Giampaolo, Rosti, Vittorio, Sabatelli, Mario, Bisogni, Giulia, Bernardo, Daniela, Luigetti, Marco, Di Paolantonio, Andrea, Guglielmino, Valeria, Romano, Angela, Nienhuis, Hans, Bulthuis-Kuiper, Janita, Kristen, Arnt V, Gerk, Olga, Ulbricht, Hannah, Taylor, Lenka, Meyle, Eva, Kleinschmidt, Natalia, Meyrath, David, Noe-Schwenn, Simone, Meng, Ulrike, Bauer, Ralf, aus dem Siepen, Fabian, Hein, Selina, Takahashi, Tetsuya, Oshita, Tomohiko, Koujin, Yoko, Neshige, Shuichiro, Nezu, Tomohisa, Segawa, Akiko, Ueno, Hiroki, Morino, Hiroyuki, Campistol, Josep M, Rodas Marin, Lida Maria, Blasco Pelicano, Josep Miquel, Dávila, Lucía Galán, Palacios, Marta, Pytel Cordoba, Vanesa, Guerrero Sola, Antonio, Horga, Alejandro, García Feijoo, Julián, Perez de Isla, Leopoldo, Marques Júnior, Wilson, Moscardini, Mariana, Litcanov, Debora Cristina, Viera Lima, Ana Flavia, Rodrigues, Leonardo, Marques Coutinho, Barbara, Moreira, Carolina Lavigne, Daccach Marques, Vanessa, Munoz Beamud, Francisco, Gragera Martínez, Álvaro, Borrachero, Cristina, Losada López, Inés Asunción, Cisneros Barroso, Eugenia, Rodríguez Rodríguez, Adrián, Sanz, Monica, Rigo Oliver, Elena, González Moreno, Juan, Gamez Martinez, Jose M, Descals, Cristina, Uson, Mercedes, Jose Vega, Francisco, Figuerola, Antoni, Montala, Carles, Waddington-Cruz, Márcia, Dias da Silva, Moises, Gervais de Santa Rosa, Renata, Pinto, Luiz Felipe, Pinto, Marcus Vinicius, Cardoso Berensztejn, Amanda, Barroso, Fabio, Lautre, Andrea, Orellana, Lucas G, González-Duarte Briseño, Maria Alejandra, Cárdenas-Soto, Karla, Jiménez López, Brenda Poled, Pérez-Castañeda, Sandra Lorena, Cantú Brito, Carlos Gerardo, Rivera de la Parra, David, Hernandez Reyes, Jose Pablo, del Mar Saniger Alba, Maria, Criollo Mora, Elia, Parman, Yesim, Rezzan, Kus Jülide, Sahin, Erdi, Serbest, Nail G, Durmus, Hacer, Cakar, Arman, Tugal Tutkun, Nuriye Ilknur, Karamursel, Sacit, Elitok, Ali, Sirin Inan, Nermin G, Altinkurt, Emre, Polydefkis, Michael, Ye, Jing, Allen, Adriane C, Chaudhry, Vinay, Jarrett, Raquel, Bressler, Neil, Burks, Kathleen L, Liu, Qingfeng, Khoshnoodi, Mohammad, Judge, Daniel P, Vista, Geno, Shah, Syed Mahmood, Hamaguchi, Hirotoshi, Oda, Junko, Fukase, Emi, Taniguchi, Ikuko, Oda, Tetsuya, Endo, Hironobu, Shimomura, Masahiro, Katanazaka, Kimitaka, Koto, Shusuke, Nakano, Takahiro, Scheid, Christof, Zueiter, Andreas, Pester, Lars, Walter, Doreen, Özdemir, Betül, Frenzel, Lukas F, Holtick, Udo, Oh, Jeeyoung, Kim, Hee Jin, Shin, Hyun Jin, Choi, Kyomin, Yamashita, Taro, Ueda, Mitsuharu, Masuda, Teruaki, Misumi, Yohei, Ueda, Akihiko, Nakahara, Keiichi, Yorita, Akiko, Tsuruhisa, Seiko, Taniwaki, Takayuki, Harada, Masaya, Moritaka, Taiga, Sakurada, Naonori, Mauricio, Elizabeth A, Baskin, Amber, Dimberg, Elliot, Dispenzieri, Angela, Fonder, Amie, Hobbs, Miriam, Russell, Stephen J, Dyck, Peter, Gonsalves, Wilson, Leung, Nelson, Witzig, Thomas E, Zeldenrust, Steven R, Hwa, Lisa, Kapoor, Prashant, Kumar, Shaji K, Lin, Yi, Lust, John A, Rajkumar, Vincent S, Dingli, David, Gertz, Morie A, Go, Ronald, Hayman, Suzanne R, Dalia, Samir, Carrillo, Esmeralda, Gorevic, Peter, Mason, Garnette, Chao, Chi-Chao, Lee, Ming-Jen, Su, Jen-Jen, Hsieh, Sung-Tsang, Tsai, Li-Kai, Yeh, Shin-Joe, Yang, Chih-Chao, Ajroud-Driss, Senda Ajroud-Driss, Casey, Patricia, Joslin, Benjamin C, Freimer, Miriam, Sankey, Alison, Kenepp, Amanda, Heintzman, Sarah, LoRusso, Samantha, Hokezu, Youichi, Kim, Byoung-Joon, Kim, JuHyeon, Lee, Ga Yeon, Cho, Eun Bin, Jeon, Eun-Seok, Min, Ju-Hong, Seok, Jin Myoung, Lee, Hye Lim, Park, Jae Hong, Sekijima, Yoshiki, Miyazawa, Chinatsu, Kato, Nagaaki, Kishida, Dai, Hineno, Akiyo, Kodaira, Minori, Yoshinaga, Tsuneaki, Miyahara, Teruyoshi, Imai, Akira, Matsumoto, Kazuhiko, Lin, Kon-Ping, Lee, Yi-Chung, Wixner, Jonas, Falk, Malin, Pilebro, Bjorn, Suhr, Ole, Lindqvist, Per, Soderberg, Karin, Pedrosa-Domellöf, Fatima, Anan, Intissar, Nordh, Erik, Tournev, Ivaylo, Zhelyazkova-Glaveeva, Sashka, Cherneva, Zheyna, Sarafov, Staiko, Chamova, Teodora, Cherninkova-Gopina, Sylvia, Schmidt, Hartmut H, Friebel, Frauke, Zibert, Andree, Mihailovic, Natasa, Schubert, Friederike, Vorona, Elena, Lahme, Larissa, Huesing-Kabar, Anna, Schilling, Matthias, Kabar, Iyad, Gillmore, Julian D, Martinez-Naharro, Ana, Chacko, Liza, Cohen, Oliver, Law, Steven, Rezk, Tamer, Lachmann, Helen J, Quan, Dianna, Blume, Brianna, Dixon, Stacy, Low, Soon Chai, Chan, Soo Looi, Lim, He Eng Li, Goh, Khean Jin, Mezei, Michelle M, Kraus, Deborah, Jack, Kristin, Wade, N. Kevin, Lopate, Glenn, Zwijack, Brittany, Florence, Julaine, Sommerville, R. Brian, Stewart, Graeme, Ryder, Julie, Mekhael, Linda, Taylor, Mark, Suan, Daniel, Wells, Karen, Stone, Paula, Itoya, Amenze, Owusu-Sekyere, Mercy, Thai, Desmond, Chahine, Ilonah, Pedrosa, Salve, Do, Thi Hoa (Therese), González-Duarte, Alejandra, Kyriakides, Theodoros, Ajroud-Driss, Senda, Mauricio, Elizabeth, Brannagan, Thomas H, III, Aldinc, Emre, Wang, Jing Jing, White, Matthew T, Vest, John, Berber, Erhan, and Sweetser, Marianne T

Published
2021
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21. CANOMAD: a neurological monoclonal gammopathy of clinical significance that benefits from B-cell–targeted therapies

Author

Le Cann, Marie, Bouhour, Françoise, Viala, Karine, Simon, Laurence, Tard, Céline, Rossi, Cédric, Morel, Guillaume, Lagrange, Emmeline, Magy, Laurent, Créange, Alain, Michaud, Maud, Franques, Jérôme, Echaniz-Laguna, Andoni, Antoine, Jean-Christophe, Baron, Marine, Arnulf, Bertrand, Puma, Angela, Delmont, Emilien, Maisonobe, Thierry, Leblond, Véronique, and Roos-Weil, Damien

Published
2020
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22. Motor unit number index as an individual biomarker: Reference limits of intra-individual variability over time in healthy subjects

Author

Delmont, Emilien, Wang, François, Lefaucheur, Jean-Pascal, Puma, Angela, Breniere, Céline, Beaudonnet, Guillemette, Cintas, Pascal, Collin, Romain, Fortanier, Etienne, Grapperon, Aude-Marie, Jomir, Laurent, Kribich, Hafida, Kouton, Ludivine, Kuntzer, Thierry, Lenglet, Timothee, Magot, Armelle, Nordine, Tarik, Ochsner, François, Bolloy, Gaëlle, Pereon, Yann, Salort-Campana, Emmanuelle, Tard, Céline, Vicino, Alex, Verschueren, Annie, and Attarian, Shahram

Published
2020
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23. Clinical, biological, electrophysiological and therapeutic profile of patients with anti-MAG neuropathy according to MYD88L265P and CXCR4 mutations and underlying haemopathy.

Author

Guérémy, Alexandre, Boucraut, José, Boudjarane, John, Grapperon, Aude-Marie, Fortanier, Etienne, Farnault, Laure, Gabert, Jean, Vely, Frédéric, Lacroix, Romaric, Kouton, Ludivine, Attarian, Shahram, and Delmont, Emilien

Subjects
BRUTON tyrosine kinase, CXCR4 receptors, SOMATIC mutation, MUCOSA-associated lymphoid tissue lymphoma, ELECTROPHYSIOLOGY
Abstract

Introduction: Anti-MAG neuropathies are associated with an IgM monoclonal gammopathy of undetermined significance (MGUS) or with a malignant haemopathy. Our objective was to determine whether the presence of a haemopathy or somatic mutations of MYD88 and CXCR4 genes influences disease presentation and response to rituximab (RTX). Methods: We included 79 patients (mean age 74 years, disease duration 9.68 years) who had a bone marrow aspiration with morphologic and immunophenotypic analysis. MYD88L265P and CXCR4 mutations were analysed in peripheral B cells. Information collected included: inflammatory neuropathy cause and treatment sensory sum score (ISS), MRC testing, overall neuropathy limitation scale (ONLS), Rash-built Overall Disability Score (RODS), ataxia score, anti-MAG titres, peak IgM dosage, neurofilament light chain levels, motor and sensory amplitudes, motor unit index (MUNIX) and motor unit size index (MUSIX) sum scores. Efficacy of RTX was evaluated at 12 months in 26 patients. Results: Malignant haematological disorders were discovered in 17 patients (22%): 13 Waldenstrom macroglobulinemia, 3 marginal zone lymphoma and one mantle cell lymphoma. MYD88L265P mutation was detected in 29/60 (48%) patients and CXCR4 in 1 single patient. Disease severity, biological and electrophysiological data and response to RTX were comparable in patients with MGUS/lymphoma and patients with/without MYD88L265P mutation. ISS was lower and MUSIX higher in patients improved by RTX. Conclusions: MYD88L265P mutation and underlying haemopathies are not predictive of a more severe disease. However, in cases of resistant and progressive neuropathy, they provide an opportunity to prescribe newly available drugs such as Bruton tyrosine kinase inhibitors. [ABSTRACT FROM AUTHOR]

Published
2024
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24. Successful autologous hematopoietic stem cell transplantation in a refractory anti‐Caspr1 antibody nodopathy.

Author

Afanasiev, Vadim, Tsouni, Pinelopi, Kuntzer, Thierry, Cairoli, Anne, Delmont, Emilien, Vallat, Jean‐Michel, Devaux, Jérôme, and Théaudin, Marie

Subjects
CHRONIC disease treatment, TREATMENT of Guillain-Barre syndrome, RITUXIMAB, METHYLPREDNISOLONE, BONE marrow transplantation, IMMUNOGLOBULINS, NEUROPHYSIOLOGY, INTRAVENOUS therapy, PLASMA exchange (Therapeutics), LYMPH nodes, IMMUNOSUPPRESSION, MOVEMENT disorders, MAGNETIC resonance imaging, MEMBRANE glycoproteins, CYCLOPHOSPHAMIDE, HEMATOPOIETIC stem cell transplantation, ATAXIA, NEURITIS
Abstract

Aim: Autoimmune nodopathies have specific clinicopathologic features, antibodies directed against nodal proteins (neurofascin 186) or paranodal proteins (neurofascin 155, contactin 1, contactin‐associated protein 1 (Caspr1)), and usually have a poor response to first‐line therapies for chronic inflammatory demyelinating polyradiculoneuropathy. Anti‐Caspr1 nodopathy treated with autologous hematopoietic stem cell transplantation (AHSCT) has not been previously reported. Methods: We report the first case of an anti‐Caspr1 antibody‐positive nodopathy refractory to high‐intensity immunosuppressive treatment, including rituximab, that responded dramatically to AHSCT. Results: A 53‐year‐old woman presented with a rapidly progressive generalized ataxic, painful motor, and inflammatory neuropathy supported by neurophysiologic and MRI studies. Initial tests for antibodies to nodal/paranodal proteins were negative. She was treated with multiple courses of intravenous immunoglobulin and methylprednisolone, plasma exchange, rituximab, and cyclophosphamide without significant clinical benefit. Repeated testing for antibodies to nodal/paranodal proteins yielded a positive result for anti‐Caspr1/IgG4 isotype antibodies. Given the poor response to multiple high intensity treatments and the relatively young age of the patient, we decided to perform AHSCT at 30 months post‐onset. Immediately after AHSCT, she stopped all immunomodulatory or immunosuppressive therapy. The Overall Neuropathy Limitation Score improved from 8/12 to 4/12 at 6 months post‐AHSCT. At 3 months post‐AHSCT, IgG4 against Caspr1 was negative and no reactivity against paranodes could be detected. Conclusion: We report a particularly severe anti‐Caspr1 antibody autoimmune nodopathy that responded dramatically to AHSCT. Although the rarity of the disease limits the possibility of larger studies, AHSCT may be a valuable therapy in treatment‐refractory cases. [ABSTRACT FROM AUTHOR]

Published
2024
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25. Antibodies against the node of Ranvier: a real-life evaluation of incidence, clinical features and response to treatment based on a prospective analysis of 1500 sera

Author

Delmont, Emilien, Brodovitch, Alexandre, Kouton, Ludivine, Allou, Thibaut, Beltran, Stéphane, Brisset, Marion, Camdessanché, Jean Philippe, Cauquil, Cécile, Cirion, Jonathan, Dubard, Thierry, Echaniz-Laguna, Andoni, Grapperon, Aude-Marie, Jauffret, Joëlle, Juntas-Morales, Raul, Kremer, Laurent Daniel, Kuntzer, Thierry, Labeyrie, Céline, Lanfranco, Lucas, Maisonobe, Thierry, Mavroudakis, Nicolas, Mecharles-Darrigol, Sylvie, Nicolas, Guillaume, Noury, Jean-Baptiste, Perie, Maud, Rajabally, Yusuf A., Remiche, Gauthier, Rouaud, Violaine, Tard, Céline, Salort-Campana, Emmanuelle, Verschueren, Annie, Viala, Karine, Wang, Adrien, Attarian, Shahram, and Boucraut, José

Published
2020
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27. Electrophysiological features of chronic inflammatory demyelinating polyradiculoneuropathy associated with IgG4 antibodies targeting neurofascin 155 or contactin 1 glycoproteins

Author

Kouton, Ludivine, Boucraut, José, Devaux, Jérome, Rajabally, Yusuf A., Adams, David, Antoine, Jean Christophe, Bourdain, Frédéric, Brodovitch, Alexandre, Camdessanché, Jean-Philippe, Cauquil, Cécile, Ciron, Jonathan, Dubard, Thierry, Echaniz-Laguna, Andoni, Grapperon, Aude-Marie, Juntas-Morales, Raul, Kremer, Laurent, Kuntzer, Thierry, Labeyrie, Céline, Lanfranco, Luca, Léger, Jean-Marc, Maisonobe, Thierry, Mavroudakis, Nicolas, Mecharles-Darrigol, Sylvie, Merle, Philippe, Noury, Jean-Baptiste, Rouaud, Violaine, Tard, Céline, Théaudin, Marie, Vallat, Jean-Michel, Viala, Karine, Attarian, Shahram, and Delmont, Emilien

Published
2020
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31. Clinical and electrophysiological characteristics of women with X‐linked Charcot–Marie–Tooth disease

Author

Barbat du Closel, Luce, primary, Bonello‐Palot, Nathalie, additional, Péréon, Yann, additional, Echaniz‐Laguna, Andoni, additional, Camdessanche, Jean Philippe, additional, Nadaj‐Pakleza, Aleksandra, additional, Chanson, Jean‐Baptiste, additional, Frachet, Simon, additional, Magy, Laurent, additional, Cassereau, Julien, additional, Cintas, Pascal, additional, Choumert, Ariane, additional, Devic, Perrine, additional, Leonard Louis, Sarah, additional, Gravier Dumonceau, Robinson, additional, Delmont, Emilien, additional, Salort‐Campana, Emmanuelle, additional, Bouhour, Françoise, additional, Latour, Philippe, additional, Stojkovic, Tanya, additional, and Attarian, Shahram, additional

Published
2023
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36. Quantification of muscle involvement by MRI in familial amyloid polyneuropathy

Author

Durelle, Clémence, Delmont, Emilien, Michel, Constance, Trabelsi, Amira, Hostin, Marc‐adrien, Ogier, Augustin C., Bendahan, David, Attarian, Shahram, APHM, CHU Timone, Centre de référence des Maladies Neuromusculaires et de la SLA, ERN-NMD, Marseille, France., Centre de résonance magnétique biologique et médicale (CRMBM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS), Institut FRESNEL (FRESNEL), Aix Marseille Université (AMU)-École Centrale de Marseille (ECM)-Centre National de la Recherche Scientifique (CNRS), and Department of Radiology, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL)

Subjects
[SDV.IB.IMA]Life Sciences [q-bio]/Bioengineering/Imaging
Published
2023

39. Validation of ELISA assays for the calculation of FLC indices for the diagnosis of intrathecal immunoglobulin synthesis

Author

Heim, Xavier, primary, Hubert, Anne Michele, additional, Raouak, Issam, additional, Nzepa, Anthony Charles, additional, Mege, Jean-Louis, additional, Delmont, Emilien, additional, Attarian, Shahram, additional, Koric, Lejla, additional, Pelletier, Jean, additional, Brodovitch, Alexandre, additional, and Boucraut, José, additional

Published
2023
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41. Contactin-1 links autoimmune neuropathy and membranous glomerulonephritis

Author

Fehmi, Janev, primary, Davies, Alexander J., additional, Antonelou, Marilina, additional, Keddie, Stephen, additional, Pikkupeura, Sonja, additional, Querol, Luis, additional, Delmont, Emilien, additional, Cortese, Andrea, additional, Franciotta, Diego, additional, Persson, Staffan, additional, Barratt, Jonathan, additional, Pepper, Ruth, additional, Farinha, Filipa, additional, Rahman, Anisur, additional, Canetti, Diana, additional, Gilbertson, Janet A., additional, Rendell, Nigel B., additional, Radunovic, Aleksandar, additional, Minton, Thomas, additional, Fuller, Geraint, additional, Murphy, Sinead M., additional, Carr, Aisling S., additional, Reilly, Mary R., additional, Eftimov, Filip, additional, Wieske, Luuk, additional, Teunissen, Charlotte E., additional, Roberts, Ian S. D., additional, Ashman, Neil, additional, Salama, Alan D., additional, and Rinaldi, Simon, additional

Published
2023
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49. Anti‐disialosyl‐immunoglobulin M chronic autoimmune neuropathies: a nationwide multicenter retrospective study

Author

Peillet, Claire, primary, Adams, David, additional, Attarian, Shahram, additional, Bouhour, Françoise, additional, Cauquil, Cécile, additional, Cassereau, Julien, additional, Chanson, Jean‐Baptiste, additional, Cintas, Pascal, additional, Creange, Alain, additional, Delmont, Emilien, additional, Fargeot, Guillaume, additional, Genestet, Steeve, additional, Gueguen, Antoine, additional, Kaminsky, Anne Laure, additional, Kuntzer, Thierry, additional, Labeyrie, Céline, additional, Michaud, Maud, additional, Pereon, Yann, additional, Puma, Angela, additional, Viala, Karine, additional, Chretien, Pascale, additional, Adam, Clovis, additional, and Echaniz‐Laguna, Andoni, additional

Published
2022
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