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45 results on '"Bruneteau, Gaelle"'

2. Caffeine consumption outcomes on amyotrophic lateral sclerosis disease progression and cognition

Author

Huin, Vincent, Blum, David, Delforge, Violette, Cailliau, Emeline, Djeziri, Sofia, Dujardin, Kathy, Genet, Alexandre, Viard, Romain, Attarian, Shahram, Bruneteau, Gaelle, Cassereau, Julien, Genestet, Steeve, Kaminsky, Anne-Laure, Soriani, Marie-Hélène, Lefilliatre, Mathilde, Couratier, Philippe, Pittion-Vouyovitch, Sophie, Esselin, Florence, De La Cruz, Elisa, Guy, Nathalie, Kolev, Ivan, Corcia, Philippe, Cintas, Pascal, Desnuelle, Claude, Buée, Luc, Danel-Brunaud, Véronique, Devos, David, and Rolland, Anne-Sophie

Published
2024
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3. Neuro-Psychological Outcome of ICU-Admitted COVID-19 Patients Presenting With CNS Complications

Author

Pelle, Juliette, Nedelec, Thomas, Marois, Clémence, Delorme, Cecile, Corvol, Jean-Christophe, Delattre, Jean-Yves, Carvalho, Stephanie, Sagnes, Sandrine, Dubois, Bruno, Navarro, Vincent, Louapre, Celine, Stojkovic, Tanya, Idbaih, Ahmed, Rosso, Charlotte, Grabli, David, Gales, Ana Zenovia, Millet, Bruno, Rohaut, Benjamin, Bayen, Eleonore, Dupont, Sophie, Bruneteau, Gaelle, Lehericy, Stephane, Seilhean, Danielle, Durr, Alexandra, Lamari, Foudil, Houot, Marion, Brochard, Vanessa Batista, Lubetzki, Catherine, Seilhean, Danielle, Pradat-Diehl, Pascale, Rosso, Charlotte, Hoang-Xuan, Khe, Fontaine, Bertrand, Naccache, Lionel, Fossati, Philippe, Arnulf, Isabelle, Durr, Alexandra, Carpentier, Alexandre, Edel, Yves, Robain, Gilberte, Thoumie, Philippe, Degos, Bertrand, Sharshar, Tarek, Alamowitch, Sonia, Apartis-Bourdieu, Emmanuelle, Peretti, Charles-Siegried, Ursu, Renata, Dzierzynski, Nathalie, Bourron, Kiyoka Kinugawa, Belmin, Joel, Oquendo, Bruno, Pautas, Eric, Verny, Marc, Samson, Yves, Leder, Sara, Leger, Anne, Deltour, Sandrine, Baronnet, Flore, Bombois, Stephanie, Touat, Mehdi, Idbaih, Ahmed, Sanson, Marc, Dehais, Caroline, Houillier, Caroline, Laigle-Donadey, Florence, Psimaras, Dimitri, Alenton, Agusti, Younan, Nadia, Villain, Nicolas, Grabli, David, del Mar Amador, Maria, Bruneteau, Gaelle, Louapre, Celine, Mariani, Louise-Laure, Mezouar, Nicolas, Mangone, Graziella, Meneret, Aurelie, Hartmann, Andreas, Tarrano, Clement, Bendetowicz, David, Pradat, Pierre-François, Baulac, Michel, Sambin, Sara, Pichit, Phintip, Chochon, Florence, Hesters, Adele, Herlin, Bastien, Nguyen, An Hung, Procher, Valerie, Demoule, Alexandre, Morawiec, Elise, Mayaux, Julien, Faure, Morgan, Ewenczyk, Claire, Coarelli, Giulia, Heinzmann, Anna, Stojkovic, Tanya, Masingue, Marion, Bassez, Guillaume, Navarro, Vincent, An, Isabelle, Worbe, Yulia, Lambrecq, Virginie, Debs, Rabab, Musat, Esteban Munoz, Lenglet, Timothee, Lambrecq, Virginie, Hanin, Aurelie, Chougar, Lydia, Shor, Nathalia, Pyatigorskaya, Nadya, Galanaud, Damien, Leclercq, Delphine, Demeret, Sophie, Rohaut, Benjamin, Cao, Albert, Marois, Clemence, Weiss, Nicolas, Gassama, Salimata, Guennec, Loic Le, Degos, Vincent, Jacquens, Alice, Similowski, Thomas, Morelot-Panzini, Capucine, Rotge, Jean-Yves, Saudreau, Bertrand, Millet, Bruno, Pitron, Victor, Sarni, Nassim, Girault, Nathalie, Maatoug, Redwan, Leu, Smaranda, Bayen, Eleonore, Thivard, Lionel, Mokhtari, Karima, Plu, Isabelle, Gonçalves, Bruno, Bottin, Laure, Yger, Marion, Ouvrard, Gaelle, Haddad, Rebecca, Ketz, Flora, Lafuente, Carmelo, Oasi, Christel, Megabarne, Bruno, Herve, Dominique, Salman, Haysam, Rametti-Lacroux, Armelle, Chalançon, Alize, Herve, Anais, Royer, Hugo, Beauzor, Florence, Maheo, Valentine, Laganot, Christelle, Minelli, Camille, Fekete, Aurelie, Grine, Abel, Biet, Marie, Hilab, Rania, Besnard, Aurore, Bouguerra, Meriem, Goudard, Gwen, Houairi, Saida, Al-Youssef, Saba, Pires, Christine, Oukhedouma, Anissa, Siuda-Krzywicka, Katarzyna, Malkinson, Tal Seidel, Agguini, Hanane, Said, Safia, and Houot, Marion

Published
2023
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4. Acoustic Change Over Time in Spastic and/or Flaccid Dysarthria in Motor Neuron Diseases

Author

Leveque, Nathalie, Slis, Anneke, Lancia, Leonardo, Bruneteau, Gaelle, and Fougeron, Cecile

Subjects
Articulation disorders -- Diagnosis -- Evaluation, Health
Abstract

Purpose: This study aims to investigate acoustic change over time as biomarkers to differentiate among spastic-flaccid dysarthria associated with amyotrophic lateral sclerosis (ALS), spastic dysarthria associated with primary lateral sclerosis (PLS), flaccid dysarthria associated with spinal and bulbar muscular atrophy (SBMA), and to explore how these acoustic parameters are affected by dysarthria severity. Method: Thirty-three ALS patients with mixed flaccid-spastic dysarthria, 17 PLS patients with pure spastic dysarthria, 18 SBMA patients with pure flaccid dysarthria, and 70 controls, all French speakers, were included in the study. Speakers produced vowel-glide sequences targeting different vocal tract shape changes. The mean and coefficient of variation of the total squared change of mel frequency cepstral coefficients were used to capture the degree and variability of acoustic changes linked to vocal tract modifications over time. Differences in duration of acoustic events were also measured. Results: All pathological groups showed significantly less acoustic change compared to controls, reflecting less acoustic contrast in sequences. Spastic and mixed spastic-flaccid dysarthric speakers showed smaller acoustic changes and slower sequence production compared to flaccid dysarthria. For dysarthria subtypes associated with a spastic component, reduced degree of acoustic change was also associated with dysarthria severity. Conclusions: The acoustic parameters partially differentiated among the dysarthria subtypes in relation to motor neuron diseases. While similar acoustic patterns were found in spastic-flaccid and spastic dysarthria, crucial differences were found between these two subtypes relating to variability. The acoustic patterns were much more variable in ALS. This method forms a promising clinical tool as a diagnostic marker of articulatory impairment, even at mild stage of dysarthria progression in all subtypes., Motor neuron diseases (MNDs) embrace a group of neurodegenerative diseases affecting lower motor neuron (LMN) and/or upper motor neuron (UMN) systems. One of the diseases that involves the LMN system [...]

Published
2022
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9. Extrapyramidal deficits in ALS: a combined biomechanical and neuroimaging study

Author

Feron, Maryse, Couillandre, Annabelle, Mseddi, Eya, Termoz, Nicolas, Abidi, Malek, Bardinet, Eric, Delgadillo, Daniel, Lenglet, Timothée, Querin, Giorgia, Welter, Marie-Laure, Le Forestier, Nadine, Salachas, François, Bruneteau, Gaelle, del Mar Amador, Maria, Debs, Rabab, Lacomblez, Lucette, Meininger, Vincent, Pélégrini-Issac, Mélanie, Bede, Peter, Pradat, Pierre-François, and de Marco, Giovanni

Published
2018
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10. Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS

Author

Miller, Timothy M, Cudkowicz, Merit E, Andrews, Jinsy A, Hesters, Adele, Kermorvant, Hugo, Lacomblez, Lucette, Forestier, Nadine Le, Lenglet, Thimotée, Retail, Maryvonne, Ruiz Del Mar Amador, Maria, Salachas, François, Shotar, Eimad, Sourour, Nader, Babu, Suma, Dorst, Johannes, Froehlich, Elke, Fromm, Andrea, Kandler, Katharina, Langer, Eva, Leichtle, Sarah, Ludolph, Albert, Mayer, Kristina, Michels, Sebastian, Raubold, Sabine, Benatar, Michael, Schuster, Joachim, Weiland, Ulrike, Wiesenfarth, Maximilian, Witzel, Simon, Calvo, Andrea, Canosa, Antonio, Casale, Federico, Chiò, Adriano, Fuda, Giuseppe, Grassano, Maurizio, McDermott, Christopher J, Marchese, Giulia, Moglia, Cristina, Palumbo, Francesca, Salamone, Paolina, Ajiki, Takahiro, Akasaka, Aya, Ando, Masahiro, Arata, Hitoshi, Asuka, Kitamura, Baba, Kosuke, Cochrane, Thos, Bekku, Goichi, Chiba, Tomoya, Date, Yugaku, Eriko, Takeuchi, Hashiguchi, Akihiro, Hatatori, Ritsuko, Hayano, Eri, Hayashi, Yuto, Higashi, Keiko, Higuchi, Eriko, Chary, Sowmya, Hiramatsu, Yu, Horikawa, Rui, Ikenaka, Kensuke, Ishiura, Hiroyuki, Ito, Daisuke, Kawai, Sachiko, Kikuchi, Junko, Kuzuyama, Haruko, Li, Xuehong, Matsumoto, Chika, Chew, Sheena, Matsuura, Eiji, Michizono, Kumiko, Mitsui, Jun, Mitsutake, Akihiko, Mochizuki, Hideki, Nagamatsu, Akemi, Nagano, Seiichi, Nakamura, Tomonori, Naruse, Hiroya, Ogasawara, Asuka, Zhu, Han, Okada, Kensuke, Okamoto, Yuji, Okuno, Tatsusada, Oyama, Satoshi, Ozono, Tatsuhiko, Sakiyama, Yusuke, Sakuishi, Kaori, Seki, Morinobu, Shibata, Shota, Shimizu, Mikito, Wu, Fan, Takahata, Katsunori, Takahito, Yoshizaki, Takashima, Hiroshi, Takeichi, Hiroko, Tashiro, Yuichi, Toda, Tatsushi, Tomizu, Yuki, Tomoya, Wadayama, Ujiakira, Nishiike, Yashita, Daiki, Nestorov, Ivan, Al-Chalabi, Ammar, Alix, James, Bangalore, Priyadarshini, Blackburn, Daniel, Chiwera, Theresa, Clegg, Rosie, Collins, Alexis, Cooper-Knock, Jonathan, Emery, Anna, Franklin, John, Genge, Angela, Graham, Danielle, Green, Louisa, Harvey, Callum, Hobson, Esther, Islam, Mahjabim, Jenkins, Thomas Michael, Kazoka, Mbombe, Kelly, Gillian, Korley, Mercy, Madarshahaian, Daniel, Mayl, Keith, Sun, Peng, McDermott, Christopher John, Radford, Alex, Shaw, Christopher, Shaw, Pamela J, Sidebottom, Joe, Smart, Lynne, Sreedharan, Jemeen, Stone, Ben, Tsironis, Theocharis, Tuddenham, Lee, McNeill, Manjit, Verber, Nick, Wollff, Helen, Young, Stacy, Zis, Panagiotis, Adamo, Ashley, Ahmed, Arubah, Ajroud-Driss, Senda, Alameda, Gustave, Arcila-Londono, Ximena, Fanning, Laura, Baird, Candy, Bazan, Tracy, Berry, James, Bordeau, Jane, Bradford, Wendy, Brook, Nyda, Brown, Lauren, Bucelli, Robert C, Ferguson, Toby A, Buckner, Katherine, Budler, Michael W, Burba, Lindita, Burke, Katherine, Calhoun, Ashley D, Campbell, Sarah, Carey, Judith, Caristo, Irys B, Carty, Simon, Chan, Emmanuel, Fradette, Stephanie, Chaudhry, Vinay, Chen, Ricky, Chow, Saephanh, Clawson, Lora L, Clemens, Mitchell, Cloninger, Suzann E, Coleman-Wood, Krista, Cooper, Thomas N, Cummings, Arlena, Daniels, Jacquelyn, VALOR, DeSaro, Pamela, DeWitt, Michelle, Dedi, Brixhilda, Dempsey, Debbie, Denny, Carol, Doherty, Jenna, Doherty, Leana, Donahue, Megan, Doyle, Michael, Duncan, Jessie, Group, OLE Working, Elman, Lauren, Eloge, Christine M, Echiti, Desirae R, Ferrey, Dominic, Fournier, Christina, Fukumura, Yuriko, Gallagher, Katherine, Garaycoa, Jessica, Garrett, Mark, Gibson, Richard L, Beullens, Lien, Gifford, Ryan, Glass, Jonathan D, Gogol, Danuta, Golden, Shea, Gonzalez, Alexa, Goodman, Ira, Goolsby, Christopher, Goslin, Kimberly, Goulbourne, Michael, Granit, Volkan, Claeys, Kristl, Grignon, Anne-Laure, GuhaRay, Adreeja, Guide, Debra, Gundogdu, Melek Betul, Gutierrez, Gil, Hastings, Debbie, Hayzen, Colleen, Herzog, Hilary, Holloway, Raegan, Jacobs, Gabriel, Claeys, Thomas, Jacobsen, Bill, James, Virginia, Jenkins, Liberty, Jockel-Balsarotti, Jennifer, Johnson, Linda Carol, Jose, Sunil, Joslin, Benjamin, Karanja, Elizabeth, Katz, Jonathan, Keener, Anthony, Couwelier, Goedele, Kittle, Gale, Klein, Sara, Kreple, Collin, Rebecca, Rebecca, Kuenzler, Kuenzler, Kusnir, Jorge, Labbe, Kristen, Lachica-Encinas, Nicolet, Ladha, Shafeeq, Leimer, Lesli, D'Hondt, Ann, Levy, Michael, Levy, Wendy, Li, Yingji, Likanje, Marie-France, Livigni, Rebecca, Locatelli, Eduardo, Luppino, Sarah, Malcolm, Amber, Maragakis, Nicholas, Marin, Horia, Debien, Elisa, Markowitz, Clyde, Markway, Jesse, McCaffrey, Alexandra, McCoy, Arita, McCoy Gross, Kelly, Mehta, Kush, Meyer, Robert, Milan, Jennifer, Miller, Timothy, Miller, Robert G, de Keersmaecker, Sebastiaan, Morales, Francisco, Mosmiller, Elizabeth, Mott, Donovan, Moulton, Kelsey, Murphy, Christine A, Negron, Tirso, Nelson, Cassandra, Newman, Daniel S, Nissinen, Janne Kristoffer, Norman, Andrew, Della Faille, Laetitia, Ohkubo, Takuya, Olney, Nicholas, Ortiz, Natasha, Oskarsson, Bjorn, Pace, Mitchell, Packard, Kathleen, Padgett, Denny, Paganoni, Sabrina, Paredes, Maria E, Parker, Elizabeth, Delmotte, Koen, Partlow, Ann, Pattee, Gary L, Paulett, Jany, Pelot, Antoinette, Pfeifer, Kyle M, Pijanowski, Olivia, Pioro, Erik, Polak, Meraida, Prakash, Ahalya, Previte, Rosemarie, Depoortere, Sofie, Pukenas, Bryan, Quinn, Colin, Ravits, John, Razavi, Ryan, Regan, Tyler, Riley, Kristen M, Roth, Heather, Sanders, Danica, Scalia, Jennifer, Schmidt, Emma, de Velder, Laura, Schwen, Edward, Shah, Jaimin, Shah, Stuti, Shefner, Jeremy, Sheldon, Danielle, Simmons, Karon, Singh, Navneet K, Singleton, Jessica, Smiley, Richard, Smith, William B, Dobbels, Laurens, Smith, Sean, Sotirchos, Elias, Sorenson, Eric, Staff, Nathan, Steele, Julie, Steijlen, Kara, Stirrat, Taylor, Stoica, George S, Strong, Stephanie, Sufit, Robert, Sobue, Gen, Gijs, Jeroen, Sultze, Jane, Swartz, Amy, Szymanski, April, Tay, Anna, Thakore, Nimish, Thiessen, Diana, Thotala, Sukrutha, Trudell, Randall G, Turcotte, Nicole, Turner, Michelle, Horckmans, Simon, Uchil, Alpa, Upadhyay, Vihar, Usman, Uzma, Vallis, Anne, Vaporean-Bussey, Danielle, Vladimirova, Valentine, Weber, Harli, Winbigler, Jennifer, Wojanowski, Heather, Wulf, Charlie, Lamaire, Nikita, Yasek, Julia, Yoo, Stephanie, Zivalic, Hannah, Cole, Alexandra, File, Greta, Foate, Jeremy, Mason, Deborah, Newton, Susan, Roberts, Stephen, Sellwood, Cory Dean, Liessens, Hannelore, Swan, James, Werno, Anja, Zhong, Cathy, Masrori, Pegah, Nysten, Celine, Schotte, Caroline, Serrien, Anouk, Swinnen, Bart, Tilkin, Petra, van Daele, Sien, Van Damme, Philip, Vynckier, Jan, Wouters, Anke, Abrahao, Agessandro, Angle, Mark, Badawy, Mohamed, Berube, Maxime, Bertone, Vanessa, Cooper, Sarah Marie, Dobrowolski, Peter, Fong, Helen, Hannouche, Matthew, Hartley, Denise, Hogan, Michael, Johnston, Wendy, Khalfallah, Yousra, Korngut, Lawrence, Kroetsch, Gina, Letourneau, Justin, Magnussen, Claire, Martinez, Jose, Massie, Rami, Mobach, Theodore, Mookshah, Jahan, Ozelsel, Timur, Parks, Andrea, Petrillo, Janet, Pfeffer, Gerald, Ludolph, Albert C, Pham, Shirley, Phung, Liane, Shiungsun, Rodney, Pi-Shan, Li, Santos, Denizart, Salmon, Kristiana, Saunders, Natalie, Sembinelli, Dylan, Tymkow, Kelsey, Wong, Berchman, Zinman, Lorne, Karlsborg, Merete, Pedersen Lomholt, Therese, Nilsson, Sigrid, Salvesen, Lisette, Skov, Pernille, Svenstrup, Kristen, Bruneteau, Gaelle, Calerencon, Frederic, and Guimaraes Costa, Raquel

Subjects
Adult, drug effects [Recovery of Function], Spinal, Oligonucleotides, blood [Neurofilament Proteins], administration & dosage [Oligonucleotides, Antisense], tofersen, Injections, blood [Amyotrophic Lateral Sclerosis], pharmacology [Oligonucleotides, Antisense], Superoxide Dismutase-1, Double-Blind Method, Neurofilament Proteins, Humans, ddc:610, Antisense, Injections, Spinal, Biomarkers, Recovery of Function, Amyotrophic Lateral Sclerosis, Oligonucleotides, Antisense, blood [Biomarkers], drug therapy [Amyotrophic Lateral Sclerosis], therapeutic use [Oligonucleotides, Antisense], SOD1 protein, human, General Medicine, genetics [Superoxide Dismutase-1], genetics [Amyotrophic Lateral Sclerosis], cerebrospinal fluid [Biomarkers], cerebrospinal fluid [Superoxide Dismutase-1], cerebrospinal fluid [Amyotrophic Lateral Sclerosis]
Abstract

The intrathecally administered antisense oligonucleotide tofersen reduces synthesis of the superoxide dismutase 1 (SOD1) protein and is being studied in patients with amyotrophic lateral sclerosis (ALS) associated with mutations in SOD1 (SOD1 ALS).In this phase 3 trial, we randomly assigned adults with SOD1 ALS in a 2:1 ratio to receive eight doses of tofersen (100 mg) or placebo over a period of 24 weeks. The primary end point was the change from baseline to week 28 in the total score on the ALS Functional Rating Scale-Revised (ALSFRS-R; range, 0 to 48, with higher scores indicating better function) among participants predicted to have faster-progressing disease. Secondary end points included changes in the total concentration of SOD1 protein in cerebrospinal fluid (CSF), in the concentration of neurofilament light chains in plasma, in slow vital capacity, and in handheld dynamometry in 16 muscles. A combined analysis of the randomized component of the trial and its open-label extension at 52 weeks compared the results in participants who started tofersen at trial entry (early-start cohort) with those in participants who switched from placebo to the drug at week 28 (delayed-start cohort).A total of 72 participants received tofersen (39 predicted to have faster progression), and 36 received placebo (21 predicted to have faster progression). Tofersen led to greater reductions in concentrations of SOD1 in CSF and of neurofilament light chains in plasma than placebo. In the faster-progression subgroup (primary analysis), the change to week 28 in the ALSFRS-R score was -6.98 with tofersen and -8.14 with placebo (difference, 1.2 points; 95% confidence interval [CI], -3.2 to 5.5; P = 0.97). Results for secondary clinical end points did not differ significantly between the two groups. A total of 95 participants (88%) entered the open-label extension. At 52 weeks, the change in the ALSFRS-R score was -6.0 in the early-start cohort and -9.5 in the delayed-start cohort (difference, 3.5 points; 95% CI, 0.4 to 6.7); non-multiplicity-adjusted differences favoring early-start tofersen were seen for other end points. Lumbar puncture-related adverse events were common. Neurologic serious adverse events occurred in 7% of tofersen recipients.In persons with SOD1 ALS, tofersen reduced concentrations of SOD1 in CSF and of neurofilament light chains in plasma over 28 weeks but did not improve clinical end points and was associated with adverse events. The potential effects of earlier as compared with delayed initiation of tofersen are being further evaluated in the extension phase. (Funded by Biogen; VALOR and OLE ClinicalTrials.gov numbers, NCT02623699 and NCT03070119; EudraCT numbers, 2015-004098-33 and 2016-003225-41.).

Published
2022

11. Development and Evaluation of a Simulation-Based Algorithm to Optimize the Planning of Interim Analyses for Clinical Trials in ALS

Author

van Unnik, Jordi W.J., primary, Nikolakopoulos, Stavros, additional, Eijkemans, Marinus J.C, additional, Gonzalez-Bermejo, Jésus, additional, Bruneteau, Gaelle, additional, Morelot-Panzini, Capucine, additional, van den Berg, Leonard H., additional, Cudkowicz, Merit E., additional, McDermott, Christopher J., additional, Similowski, Thomas, additional, and van Eijk, Ruben P.A., additional

Published
2023
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12. C9ORF72 knockdown triggers FTD-like symptoms and cell pathology in mice

Author

Lopez-Herdoiza, Maria-Belen, primary, Bauché, Stephanie, additional, Wilmet, Baptiste, additional, Le Duigou, Caroline, additional, Roussel, Delphine, additional, Frah, Magali, additional, Béal, Jonas, additional, Devely, Gabin, additional, Boluda, Susana, additional, Frick, Petra, additional, Bouteiller, Delphine, additional, Dussaud, Sébastien, additional, Guillabert, Pierre, additional, Dalle, Carine, additional, Dumont, Magali, additional, Camuzat, Agnes, additional, Saracino, Dario, additional, Barbier, Mathieu, additional, Bruneteau, Gaelle, additional, Ravassard, Phillippe, additional, Neumann, Manuela, additional, Nicole, Sophie, additional, Le Ber, Isabelle, additional, Brice, Alexis, additional, and Latouche, Morwena, additional

Published
2023
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15. Muscle cells of sporadic amyotrophic lateral sclerosis patients secrete neurotoxic vesicles

Author

Le Gall, Laura, primary, Duddy, William J., additional, Martinat, Cecile, additional, Mariot, Virginie, additional, Connolly, Owen, additional, Milla, Vanessa, additional, Anakor, Ekene, additional, Ouandaogo, Zamalou G., additional, Millecamps, Stephanie, additional, Lainé, Jeanne, additional, Vijayakumar, Udaya Geetha, additional, Knoblach, Susan, additional, Raoul, Cedric, additional, Lucas, Olivier, additional, Loeffler, Jean Philippe, additional, Bede, Peter, additional, Behin, Anthony, additional, Blasco, Helene, additional, Bruneteau, Gaelle, additional, Del Mar Amador, Maria, additional, Devos, David, additional, Henriques, Alexandre, additional, Hesters, Adele, additional, Lacomblez, Lucette, additional, Laforet, Pascal, additional, Langlet, Timothee, additional, Leblanc, Pascal, additional, Le Forestier, Nadine, additional, Maisonobe, Thierry, additional, Meininger, Vincent, additional, Robelin, Laura, additional, Salachas, Francois, additional, Stojkovic, Tanya, additional, Querin, Giorgia, additional, Dumonceaux, Julie, additional, Butler Browne, Gillian, additional, González De Aguilar, Jose‐Luis, additional, Duguez, Stephanie, additional, and Pradat, Pierre Francois, additional

Published
2022
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17. Muscle cells of sporadic ALS patients secrete neurotoxic vesicles

Author

Gall, Laura Le, primary, Duddy, William J, additional, Martinat, Cecile, additional, Mariot, Virginie, additional, Connolly, Owen, additional, Milla, Vanessa, additional, Anakor, Ekene, additional, Ouandaogo, Zamalou G, additional, Millecamps, Stephanie, additional, Lainé, Jeanne, additional, Vijayakumar, Udaya Geetha, additional, Knoblach, Susan, additional, Raoul, Cedric, additional, Lucas, Olivier, additional, Loeffler, Jean Philippe, additional, Bede, Peter, additional, Behin, Anthony, additional, Blasco, Helene, additional, Bruneteau, Gaelle, additional, Del Mar Amador, Maria, additional, Devos, David, additional, Henriques, Alexandre, additional, Hesters, Adele, additional, Lacomblez, Lucette, additional, Laforet, Pascal, additional, Langlet, Timothee, additional, Leblanc, Pascal, additional, Le Forestier, Nadine, additional, Maisonobe, Thierry, additional, Meininger, Vincent, additional, Robelin, Laura, additional, Salachas, Francois, additional, Stojkovic, Tanya, additional, Querin, Giorgia, additional, Dumonceaux, Julie, additional, Browne, Gillian Butler, additional, González De Aguilar, Jose-Luis, additional, Duguez, Stephanie, additional, and Pradat, Pierre Francois, additional

Published
2021
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19. Phenotype difference between ALS patients with expanded repeats in C9ORF72 and patients with mutations in other ALS-related genes

Author

Millecamps, Stéphanie, Boillée, Séverine, Le Ber, Isabelle, Seilhean, Danielle, Teyssou, Elisa, Giraudeau, Marine, Moigneu, Carine, Vandenberghe, Nadia, Danel-Brunaud, Véronique, Corcia, Philippe, Pradat, Pierre-François, Le Forestier, Nadine, Lacomblez, Lucette, Bruneteau, Gaelle, Camu, William, Brice, Alexis, Cazeneuve, Cécile, LeGuern, Eric, Meininger, Vincent, and Salachas, François

Published
2012
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20. Implanted Phrenic Stimulation Impairs Local Diaphragm Myofiber Reinnervation in Amyotrophic Lateral Sclerosis

Author

Guimarães-Costa, Raquel, primary, Niérat, Marie-Cécile, additional, Rivals, Isabelle, additional, Morélot-Panzini, Capucine, additional, Romero, Norma Beatriz, additional, Menegaux, Fabrice, additional, Salachas, François, additional, Gonzalez-Bermejo, Jésus, additional, Similowski, Thomas, additional, Bruneteau, Gaëlle, additional, del Mar Amador, Maria, additional, Antoine, Jean Christophe, additional, Arne-Bes, Marie Christine, additional, Attali, Valérie, additional, Beauvais, Katell, additional, Brunaud-Danel, Veronique, additional, Bruneteau, Gaelle, additional, Camdessanche, Jean-Philippe, additional, Camu, William, additional, Carluer, Laurence, additional, Cassereau, Julien, additional, Chapart, Maud, additional, Chenuel, Bruno, additional, Chereau, Nathalie, additional, Cintas, Pascal, additional, Clavelou, Pierre, additional, Corcia, Philippe, additional, Cornette, André, additional, Couratier, Philippe, additional, Court-Fortune, Isabelle, additional, Cuvelier, Antoine, additional, Delclaux, Christophe, additional, Desnuelle, Claude, additional, Di Maria, Sophie, additional, Fargeot, Catherine, additional, Fauroux, Brigitte, additional, Finet-Monnier, Armelle, additional, Fleury, Marie Celine, additional, Gaillat, Carmen, additional, Georges, Marjolaine, additional, Giroud, Maurice, additional, Gonzalez-Bermejo, Jesus, additional, Greil, Annick, additional, Guerrier, Muriel, additional, Guillaume, Nicolas, additional, Guy, Nathalie, additional, Hannequin, Didier, additional, Juillien, Valérie, additional, Juntas-Morales, Raul, additional, Kessler, Romain, additional, Kolev, Yvan, additional, Lagarde, Julien, additional, Lautrette, Geraldine, additional, Le Cam, Pierre, additional, LeForestier, Nadine, additional, Lemasson, Gwendal, additional, Lenglet, Thimothée, additional, Magro, Pascal, additional, Mallart, Anne, additional, Martinaud, Olivier, additional, Meininger, Vincent, additional, Meslier, Nicole, additional, Moirot, Pierre, additional, Morelot-Panzini, Capucine, additional, Nadjar, Yann, additional, Niel-Duriez, Myriam, additional, Nierat, Marie-Cecile, additional, Noullet, Severine, additional, Pageot, Nicolas, additional, Perez, Thierry, additional, Perrin, Christophe, additional, Pillet, Odile, additional, Pittion, Sophie, additional, Pouget, Jean, additional, Pradat, Pierre-François, additional, Prigent, Hélène, additional, Rabec, Claudio, additional, Ricard, Jean-Damien, additional, Romero, Norma, additional, Royer, Catherine, additional, Schaup, Barbara, additional, Sedkaoui, Kamila, additional, Soriani, Marie-Hélène, additional, Straus, Christian, additional, Tanguy, Marie-Laure, additional, Tranchant, Christine, additional, Vandenberghue, Nadia, additional, Vershueren, Annie, additional, Viader, Fausto, additional, and Wienalek-Bachelet, Anne-Cecile, additional

Published
2019
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22. SOD1, ANG, VAPB, TARDBP, and FUS mutations in familial amyotrophic lateral sclerosis: genotype-phenotype correlations

Author

Millecamps, Stephanie, Salachas, Francois, Cazeneuve, Cecile, Gordon, Paul, Bricka, Bernard, Camuzat, Agnes, Guillot-Noel, Lena, Russaouen, Odile, Bruneteau, Gaelle, Pradat, Pierre-Francois, Le Forestier, Nadine, Vandenberghe, Nadia, Danel-Brunaud, Veronique, Guy, Nathalie, Thauvin-Robinet, Christel, Lacomblez, Lucette, Couratier, Philippe, Hannequin, Didier, Seilhean, Danielle, Le Ber, Isabelle, Corcia, Philippe, Camu, William, Brice, Alexis, Rouleau, Guy, LeGuern, Eric, and Meininger, Vincent

Subjects
Amyotrophic lateral sclerosis -- Genetic aspects, Amyotrophic lateral sclerosis -- Development and progression, Amyotrophic lateral sclerosis -- Research, Gene mutations -- Identification and classification, Gene mutations -- Research, Health
Published
2010

24. Mutations in UBQLN2 are rare in French amyotrophic lateral sclerosis

Author

Millecamps, Stéphanie, Corcia, Philippe, Cazeneuve, Cécile, Boillée, Séverine, Seilhean, Danielle, Danel-Brunaud, Véronique, Vandenberghe, Nadia, Pradat, Pierre-François, Le Forestier, Nadine, Lacomblez, Lucette, Bruneteau, Gaëlle, Camu, William, Brice, Alexis, Meininger, Vincent, LeGuern, Eric, and Salachas, François

Published
2012
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25. Cursive Eye-Writing With Smooth-Pursuit Eye-Movement Is Possible in Subjects With Amyotrophic Lateral Sclerosis

Author

Lenglet, Timothée, primary, Mirault, Jonathan, additional, Veyrat-Masson, Marie, additional, Funkiewiez, Aurélie, additional, Amador, Maria del Mar, additional, Bruneteau, Gaelle, additional, Le Forestier, Nadine, additional, Pradat, Pierre-Francois, additional, Salachas, Francois, additional, Vacher, Yannick, additional, Lacomblez, Lucette, additional, and Lorenceau, Jean, additional

Published
2019
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26. Screening of OPTN in French familial amyotrophic lateral sclerosis

Author

Millecamps, Stéphanie, Boillée, Séverine, Chabrol, Elodie, Camu, William, Cazeneuve, Cécile, Salachas, François, Pradat, Pierre-François, Danel-Brunaud, Véronique, Vandenberghe, Nadia, Corcia, Philippe, Le Forestier, Nadine, Lacomblez, Lucette, Bruneteau, Gaëlle, Seilhean, Danielle, Brice, Alexis, Feingold, Josué, Meininger, Vincent, and LeGuern, Eric

Published
2011
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28. Safety and efficacy of ozanezumab in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled, phase 2 trial

Author

Meininger, Vincent, primary, Genge, Angela, additional, van den Berg, Leonard H, additional, Robberecht, Wim, additional, Ludolph, Albert, additional, Chio, Adriano, additional, Kim, Seung H, additional, Leigh, P Nigel, additional, Kiernan, Matthew C, additional, Shefner, Jeremy M, additional, Desnuelle, Claude, additional, Morrison, Karen E, additional, Petri, Susanne, additional, Boswell, Diane, additional, Temple, Jane, additional, Mohindra, Rajat, additional, Davies, Matt, additional, Bullman, Jonathan, additional, Rees, Paul, additional, Lavrov, Arseniy, additional, Abdulla, Susanne, additional, Alsop, Cathy, additional, Barbieri, Francesca, additional, Bates, Stewart, additional, Berry, James D, additional, Botez, Stephan A, additional, Bruneteau, Gaelle, additional, Calvo, Andrea, additional, Camejo, Rodrigo Refoios, additional, Camu, William, additional, Chauhan, Deven, additional, Danel-Brunaud, Veronique, additional, Daniluk, Jerzy, additional, Dekker, Annelot, additional, Destee, Alain, additional, Devine, Matthew, additional, DeWall, Stephen, additional, Dorst, Johannes, additional, Fuda, Giuseppe, additional, Fujimura, Harutoshi, additional, Funke, Andreas, additional, Grehl, Torsten, additional, Grosskreutz, Julian, additional, Gungabissoon, Usha, additional, Henderson, Robert, additional, Ho, Peggy, additional, Huynh, William, additional, Jacob, Saiju, additional, Juntas-Morales, Raul, additional, Kim, Byung-Jo, additional, Kobeleva, Xenia, additional, Koerner, Sonja, additional, Kolb, Stephen, additional, Kollewe, Katja, additional, Korngut, Lawrence, additional, Lautrette, Geraldine, additional, Lee, Amy, additional, Lynch, Anthony, additional, Massie, Rami, additional, Matte, Genevieve, additional, Menezes, Darryl, additional, Milleri, Stefano, additional, Nichols, Linda, additional, Nishiyama, Kazutoshi, additional, Ogino, Mieko, additional, Parkinson, Chris, additional, Pradat, Pierre-François, additional, Prell, Tino, additional, Price, Jeffrey, additional, Ramsey, Eleanor, additional, Ringer, Thomas M, additional, Salmon, Kristiana, additional, Shoesmith, Christen, additional, Soriani, Marie Helene, additional, Stam, Marloes, additional, Steinberg, Erik, additional, Stubbs, Rob, additional, Sullivan, Herman, additional, Van Damme, Philip, additional, van Es, Michael, additional, Visser, Anne, additional, Watson, Mary Lou, additional, Winkler, Andrea Sylvia, additional, Zinman, Lorne, additional, and Zoing, Margie, additional

Published
2017
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29. Correction: Cervical Spinal Cord Atrophy Profile in Adult SMN1-Linked SMA

Author

El Mendili, Mohamed-Mounir, primary, Lenglet, Timothée, additional, Stojkovic, Tanya, additional, Behin, Anthony, additional, Guimarães-Costa, Raquel, additional, Salachas, François, additional, Meininger, Vincent, additional, Bruneteau, Gaelle, additional, Le Forestier, Nadine, additional, Laforêt, Pascal, additional, Lehéricy, Stéphane, additional, Benali, Habib, additional, and Pradat, Pierre-François, additional

Published
2016
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30. Endplate denervation correlates with Nogo-A muscle expression in amyotrophic lateral sclerosis patients

Author

Bruneteau, Gaelle, Bauché, Stéphanie, Gonzalez De Aguilar, Jose-Luis, Brochier, Guy, Mandjee, Nathalie, Tanguy, Marie-Laure, Hussain, Ghulam, Behin, Anthony, Khiami, Frédéric, Sariali, El Hadi, Hell-Remy, Caroline, Salachas, François, Pradat, Pierre-François, Lacomblez, Lucette, Nicole, Sophie, Fontaine, Bertrand, Fardeau, Michel, Loeffler, Jean-Philippe, Meininger, Vincent, Fournier, Emmanuel, Koenig, Jeanine, Hantai, Daniel, CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute (ICM), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Centre de recherche en Myologie – U974 SU-INSERM, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Universidad de Córdoba = University of Córdoba [Córdoba], Institut de Myologie, Université Pierre et Marie Curie - Paris 6 (UPMC)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Association française contre les myopathies (AFM-Téléthon)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Mécanismes Centraux et Périphériques de la Neurodégénérescence, Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire d'Imagerie Biomédicale [Paris] (LIB), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université Pierre et Marie Curie - Paris 6 (UPMC), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), and Administateur, HAL Sorbonne Université

Subjects
[SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, nervous system, [SDV]Life Sciences [q-bio], Research Articles, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

International audience; ObjectiveData from mouse models of amyotrophic lateral sclerosis (ALS) suggest early morphological changes in neuromuscular junctions (NMJs), with loss of nerve–muscle contact. Overexpression of the neurite outgrowth inhibitor Nogo-A in muscle may play a role in this loss of endplate innervation.MethodsWe used confocal and electron microscopy to study the structure of the NMJs in muscle samples collected from nine ALS patients (five early-stage patients and four long-term survivors). We correlated the morphological results with clinical and electrophysiological data, and with Nogo-A muscle expression level.ResultsSurface electromyography assessment of neuromuscular transmission was abnormal in 3/9 ALS patients. The postsynaptic apparatus was morphologically altered for almost all NMJs (n = 430) analyzed using confocal microscopy. 19.7% of the NMJs were completely denervated (fragmented synaptic gutters and absence of nerve terminal profile). The terminal axonal arborization was usually sparsely branched and 56.8% of innervated NMJs showed a typical reinnervation pattern. Terminal Schwann cell (TSC) morphology was altered with extensive cytoplasmic processes. A marked intrusion of TSCs in the synaptic cleft was seen in some cases, strikingly reducing the synaptic surface available for neuromuscular transmission. Finally, high-level expression of Nogo-A in muscle was significantly associated with higher extent of NMJ denervation and negative functional outcome.InterpretationOur results support the hypothesis that morphological alterations of NMJs are present from early-stage disease and may significantly contribute to functional motor impairment in ALS patients. Muscle expression of Nogo-A is associated with NMJ denervation and thus constitutes a therapeutic target to slow disease progression.

Published
2015

31. Cervical Spinal Cord Atrophy Profile in Adult SMN1-Linked SMA

Author

El Mendili, Mohamed-Mounir, primary, Lenglet, Timothée, additional, Stojkovic, Tanya, additional, Behin, Anthony, additional, Guimarães-Costa, Raquel, additional, Salachas, François, additional, Meininger, Vincent, additional, Bruneteau, Gaelle, additional, Le Forestier, Nadine, additional, Laforêt, Pascal, additional, Lehéricy, Stéphane, additional, Benali, Habib, additional, and Pradat, Pierre-François, additional

Published
2016
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32. Validation of robust tools to measure sialorrhea in amyotrophic lateral sclerosis: A study in a large French cohort

Author

Abdelnour-Mallet, Maya, primary, Tezenas Du Montcel, Sophie, additional, Cazzolli, Pamela A., additional, Assouline, Avi, additional, Pointon, Christine, additional, Leveque, Nathalie, additional, Dominique, Henri, additional, Elmazria, Hassan, additional, Rothmayer, Michaela, additional, Lenglet, Timothee, additional, Bruneteau, Gaelle, additional, le Forestier, Nadine, additional, Delanian, Sylvie, additional, Gonzalez-Bermejo, Jesus, additional, Salachas, François, additional, Brooks, Benjamin Rix, additional, and Pradat, Pierre François, additional

Published
2012
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34. Phenotype difference between ALS patients with expanded repeats inC9ORF72and patients with mutations in other ALS-related genes

Author

Millecamps, Stéphanie, primary, Boillée, Séverine, additional, Le Ber, Isabelle, additional, Seilhean, Danielle, additional, Teyssou, Elisa, additional, Giraudeau, Marine, additional, Moigneu, Carine, additional, Vandenberghe, Nadia, additional, Danel-Brunaud, Véronique, additional, Corcia, Philippe, additional, Pradat, Pierre-François, additional, Le Forestier, Nadine, additional, Lacomblez, Lucette, additional, Bruneteau, Gaelle, additional, Camu, William, additional, Brice, Alexis, additional, Cazeneuve, Cécile, additional, LeGuern, Eric, additional, Meininger, Vincent, additional, and Salachas, François, additional

Published
2012
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36. Abnormalities of satellite cells function in amyotrophic lateral sclerosis

Author

Pradat, Pierre-François, primary, Barani, Aude, additional, Wanschitz, Julia, additional, Dubourg, Odile, additional, Lombès, Anne, additional, Bigot, Anne, additional, Mouly, Vincent, additional, Bruneteau, Gaelle, additional, Salachas, François, additional, Lenglet, Timothée, additional, Meininger, Vincent, additional, and Butler-Browne, Gillian, additional

Published
2011
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37. Safety of home parenteral nutrition in patients with amyotrophic lateral sclerosis: A French national survey

Author

Abdelnour-Mallet, Maya, primary, Verschueren, Annie, additional, Guy, Nathalie, additional, Soriani, Marie-Hélène, additional, Chalbi, Myriam, additional, Gordon, Paul, additional, Salachas, François, additional, Bruneteau, Gaelle, additional, le Forestier, Nadine, additional, Lenglet, Timothée, additional, Desnuelle, Claude, additional, Clavelou, Pierre, additional, Pouget, Jean, additional, Meininger, Vincent, additional, and Pradat, Pierre-François, additional

Published
2010
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38. Impaired glucose tolerance in patients with amyotrophic lateral sclerosis

Author

Pradat, Pierre-Francois, primary, Bruneteau, Gaelle, additional, Gordon, Paul H., additional, Dupuis, Luc, additional, Bonnefont-Rousselot, Dominique, additional, Simon, Dominique, additional, Salachas, Francois, additional, Corcia, Philippe, additional, Frochot, Vincent, additional, Lacorte, Jean-Marc, additional, Jardel, Claude, additional, Coussieu, Christiane, additional, Le Forestier, Nadine, additional, Lacomblez, Lucette, additional, Loeffler, Jean-Philippe, additional, and Meininger, Vincent, additional

Published
2010
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39. Impaired glucose tolerance in patients with amyotrophic lateral sclerosis

Author

Pradat, Pierre-Francois, primary, Bruneteau, Gaelle, additional, Gordon, Paul, additional, Dupuis, Luc, additional, Bonnefont-Rousselot, Dominique, additional, Simon, Dominique, additional, Salachas, Francois, additional, Corcia, Philippe, additional, Frochot, Vincent, additional, Lacorte, Jean-Marc, additional, Jardel, Claude, additional, Coussieu, Christiane, additional, Forestier, Nadine Le, additional, Lacomblez, Lucette, additional, Loeffler, Jean-Philippe, additional, and Meininger, Vincent, additional

Published
2009
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40. Reply

Author

Pradat, Pierre-François, primary, Gonzalez de Aguilar, Jose-Luis, additional, Bruneteau, Gaelle, additional, Dupuis, Luc, additional, Echaniz-Laguna, Andoni, additional, Loeffler, Jean-Philippe, additional, and Meininger, Vincent, additional

Published
2007
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41. Muscle Nogo-a expression is a prognostic marker in lower motor neuron syndromes

Author

Pradat, Pierre-François, primary, Bruneteau, Gaelle, additional, Gonzalez de Aguilar, Jose-Luis, additional, Dupuis, Luc, additional, Jokic, Natasa, additional, Salachas, François, additional, Le Forestier, Nadine, additional, Echaniz-Laguna, Andoni, additional, Dubourg, Odile, additional, Hauw, Jean-Jacques, additional, Tranchant, Christine, additional, Loeffler, Jean-Philippe, additional, and Meininger, Vincent, additional

Published
2007
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42. Validation of robust tools to measure sialorrhea in amyotrophic lateral sclerosis: A study in a large French cohort.

Author

Abdelnour-Mallet, Maya, Tezenas Du Montcel, Sophie, Cazzolli, Pamela A., Assouline, Avi, Pointon, Christine, Leveque, Nathalie, Dominique, Henri, Elmazria, Hassan, Rothmayer, Michaela, Lenglet, Timothee, Bruneteau, Gaelle, le Forestier, Nadine, Delanian, Sylvie, Gonzalez-Bermejo, Jesus, Salachas, François, Brooks, Benjamin Rix, and Pradat, Pierre François

Subjects
DROOLING, AMYOTROPHIC lateral sclerosis, NEUROLOGISTS, COMPARATIVE studies, SALIVATION, COHORT analysis
Abstract

There is an unmet need for validated tools to measure sialorrhea in amyotrophic lateral sclerosis, especially to evaluate treatments. We assessed the inter-/intra-rate reviewer reliability of two scales: the Oral Secretion Scale (OSS), specifically developed for ALS patients, and the Sialorrhea Scoring Scale (SSS), initially developed for Parkinson's disease patients. Sialorrhea was rated in 69 ALS consecutive patients by four evaluators: two neurologists, one nurse and one speech therapist. Inter-rater reliability was evaluated by the light kappa coefficient and intra-rater reliability by the weighted kappa coefficient. We also compared patients' and caregivers' answers. Results demonstrated that the two scales present a high inter-/intra-rater reliability: weighted kappas were 0.85 for both scales and light kappas 0.89 for the OSS and 0.88 for the SSS. Both scales also showed a good intra-profession reliability (OSS kappa = 0.84; SSS kappa = 0.79) and agreement between patients' and caregivers' answers. The SSS showed a higher responsiveness compared to OSS. In conclusion, both Oral Secretion Scale and Sialorrhea Scoring Scale are reliable tools to measure sialorrhea in ALS patients. Because of the wide range of salivation degrees, SSS may be more sensitive as a tool to evaluate treatments in patients with severe hypersialorrhea. [ABSTRACT FROM AUTHOR]

Published
2013
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43. Predicting Survival of Patients with Amyotrophic Lateral Sclerosis at Presentation: A 15-Year Experience.

Author

Gordon, Paul H., Salachas, François, Lacomblez, Lucette, Le Forestier, Nadine, Pradat, Pierre-François, Bruneteau, Gaelle, Elbaz, alexis, and Meininger, Vincent

Subjects
AMYOTROPHIC lateral sclerosis, MUSCLE strength, NEUROMUSCULAR diseases, MOTOR neuron diseases, EPILEPSY, NEUROLOGY
Abstract

Objective: To describe the clinical features at first evaluation that best predict survival of the amyotrophic lateral sclerosis (ALS) population from the Salpêtrière Hospital between 1995 and 2009. Methods: Data are collected and entered into a clinical database from all patients seen at the Paris ALS Center. Variables analyzed were demographic and baseline information, strength testing (manual muscle testing; 1995-2009), the revised ALS Functional Rating Scale (ALSFRS-R; 2002-2009) and survival status. The χ2 test and ANOVA assessed differences in variables by region and across time period. Univariate and multivariate Cox proportional hazards models determined which variables best predicted survival. Flexible modeling of continuous predictors (splines) assessed trends in survival for different variables. Results: 3,885 patients with ALS were seen in 1995-2009, of whom 2,037 had ALSFRS-R scores. Age, weight, strength, and site of onset varied by region of residence. The proportion of patients living outside Paris, the time to first visit, patient age, and motor function differed across time periods. In Cox models, site of onset, time to first visit greater than 18 months, strength and the year of visit after 2006 predicted survival (all p values <0.0001). Compared to patients first seen between 1999 and 2002, the hazard ratio of death was 1.04 (95% CI = 0.95-1.14) for 2003-2006, and 0.76 (95% CI = 0.66-0.87) after 2006, while adjusting for other predictors of survival. The use of noninvasive ventilation increased during 2004-2008 from 16 to 51% of patients. Conclusions: Older age, bulbar onset, shorter delay to first visit and poor motor function at first visit predicted shorter survival rates in this large center-based sample from France, showing marked consistency across time and region of residence. Survival improved after 2006, concurrent with increasing rates of noninvasive ventilation use. Clinicopathologic correlation could better define subgroups, but identification of etiologies may be needed to elucidate individual forms of ALS with unique survival patterns. Copyright © 2012 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Published
2013
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44. Safety of home parenteral nutrition in patients with amyotrophic lateral sclerosis: A French national survey.

Author

Abdelnour-Mallet, Maya, Verschueren, Annie, Guy, Nathalie, Soriani, Marie-Hélène, Chalbi, Myriam, Gordon, Paul, Salachas, François, Bruneteau, Gaelle, le Forestier, Nadine, Lenglet, Timothée, Desnuelle, Claude, Clavelou, Pierre, Pouget, Jean, Meininger, Vincent, and Pradat, Pierre-François

Subjects
PARENTERAL feeding, AMYOTROPHIC lateral sclerosis, SURVEYS, HOME care services, CENTRAL venous catheters, CONFIDENCE intervals, PATIENTS
Abstract

We carried out a retrospective multicentre study to assess the safety of home parenteral nutrition (HPN) in patients with ALS. We reviewed the case records of patients from French ALS centres treated with HPN by central venous catheter (CVC) using an implantable port between January 2005 and October 2009. Seventy-three patients received HPN for a total of 11,908 catheter days. Twenty-seven patients experienced a total of 37 CVC related complications resulting in an incidence rate of 3.11 CVC complications/1000 catheter days, including 1.93 septic complications and 1.09 mechanical complications/1000 catheter days. Metabolic complications were frequent but without serious consequences on mortality. The use of the catheter for intravenous therapies in addition to HPN was identified as a septicaemia's risk factor (relative risk (RR) == 2.54, confidence interval (CI) 1.56--4.14, p == 0.04). In conclusion, HPN is an alternative procedure to PEG in advanced ALS patients. The incidence of complications appears to be comparable to data from the literature on HPN in other diseases. A prospective study comparing HPN and radiologic inserted gastrostomy (RIG) would allow comparison of the relative risk-benefit and survival of these procedures. The relation of CVC and RIG placement timing and the complications'' occurrence should also be investigated. [ABSTRACT FROM AUTHOR]

Published
2011
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