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48 results on '"Bruneteau, Gaëlle"'

1. Effect of ultrasound-mediated blood-spinal cord barrier opening on survival and motor function in females in an amyotrophic lateral sclerosis mouse model

Author: Montero, Anne-Sophie, Aliouat, Ilyes, Ribon, Matthieu, Canney, Michael, Goldwirt, Lauriane, Mourah, Samia, Berriat, Félix, Lobsiger, Christian S., Pradat, Pierre-François, Salachas, François, Bruneteau, Gaëlle, Carpentier, Alexandre, and Boillée, Séverine
Published: 2024
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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. Acoustic Change over Time in Spastic and/or Flaccid Dysarthria in Motor Neuron Diseases

Author: Lévêque, Nathalie, Slis, Anneke, Lancia, Leonardo, Bruneteau, Gaëlle, and Fougeron, Cécile
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.
Published: 2022
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4. Development of new outcome measures for adult SMA type III and IV: a multimodal longitudinal study

Author: Querin, Giorgia, Lenglet, Timothée, Debs, Rabab, Stojkovic, Tanya, Behin, Anthony, Salachas, François, Le Forestier, Nadine, Amador, Maria Del Mar, Bruneteau, Gaëlle, Laforêt, Pascal, Blancho, Sophie, Marchand-Pauvert, Véronique, Bede, Peter, Hogrel, Jean-Yves, and Pradat, Pierre-François
Published: 2021
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5. Survival of amyotrophic lateral sclerosis patients after admission to the intensive care unit for acute respiratory failure: an observational cohort study

Author: Mayaux, Julien, Lambert, Jérôme, Morélot-Panzini, Capucine, Gonzalez-Bermejo, Jésus, Delemazure, Julie, Llontop, Claudia, Bruneteau, Gaëlle, Salachas, François, Dres, Martin, Demoule, Alexandre, and Similowski, Thomas
Published: 2019
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6. The multidimensional nature of dyspnoea in amyotrophic lateral sclerosis patients with chronic respiratory failure: Air hunger, anxiety and fear

Author: Morélot-Panzini, Capucine, Perez, Thierry, Sedkaoui, Kamila, de Bock, Elodie, Aguilaniu, Bernard, Devillier, Philippe, Pignier, Christophe, Arnould, Benoit, Bruneteau, Gaëlle, and Similowski, Thomas
Published: 2018
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7. The motor unit number index (MUNIX) profile of patients with adult spinal muscular atrophy

Author: Querin, Giorgia, Lenglet, Timothée, Debs, Rabab, Stojkovic, Tanya, Behin, Anthony, Salachas, François, Le Forestier, Nadine, Amador, Maria del Mar, Lacomblez, Lucette, Meininger, Vincent, Bruneteau, Gaelle, Laforêt, Pascal, Blancho, Sophie, Marchand-Pauvert, Véronique, Bede, Peter, Hogrel, Jean-Yves, and Pradat, Pierre-François
Published: 2018
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8. Predictive factors for prognosis after gastrostomy placement in routine non-invasive ventilation users ALS patients

Author: Hesters, Adèle, Amador, Maria del Mar, Debs, Rabab, Le Forestier, Nadine, Lenglet, Timothée, Pradat, Pierre-François, Salachas, François, Faure, Morgane, Jimenez, Maria-Alejandra Galarza, Gonzalez-Bermejo, Jesus, Morelot, Capucine, and Bruneteau, Gaëlle
Published: 2020
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9. Unusual association of amyotrophic lateral sclerosis and myasthenia gravis: A dysregulation of the adaptive immune system?

Author: del Mar Amador, Maria, Vandenberghe, Nadia, Berhoune, Nawel, Camdessanché, Jean-Philippe, Gronier, Sophie, Delmont, Emilien, Desnuelle, Claude, Cintas, Pascal, Pittion, Sophie, Louis, Sarah, Demeret, Sophie, Lenglet, Timothée, Meininger, Vincent, Salachas, François, Pradat, Pierre-François, and Bruneteau, Gaëlle
Published: 2016
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10. Radiation Therapy for Hypersalivation: A Prospective Study in 50 Amyotrophic Lateral Sclerosis Patients

Author: Assouline, Avi, Levy, Antonin, Abdelnour-Mallet, Maya, Gonzalez-Bermejo, Jesus, Lenglet, Timothée, Le Forestier, Nadine, Salachas, François, Bruneteau, Gaelle, Meininger, Vincent, Delanian, Sylvie, and Pradat, Pierre-François
Published: 2014
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11. Acoustic Change Over Time in Spastic and/or Flaccid Dysarthria in Motor Neuron Diseases

Author: Lévêque, Nathalie, primary, Slis, Anneke, additional, Lancia, Leonardo, additional, Bruneteau, Gaëlle, additional, and Fougeron, Cécile, additional
Published: 2022
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12. Traitement de la sialorrhée dans la sclérose latérale amyotrophique et la maladie de Parkinson par radiothérapie des glandes salivaires : analyse de 281 cas

Author: Assouline, Avi, primary, Schernberg, Antoine, additional, Del Mar Amador, Maria, additional, Le Forestier, Nadine, additional, Bruneteau, Gaëlle, additional, Salachas, François, additional, and Pradat, Pierre-François, additional
Published: 2022
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13. The wide spectrum of COVID-19 neuropsychiatric complications within a multidisciplinary centre

Author: Delorme, Cécile, Houot, Marion, Rosso, Charlotte, Carvalho, Stéphanie, Nedelec, Thomas, Maatoug, Redwan, Pitron, Victor, Gassama, Salimata, Sambin, Sara, Bombois, Stéphanie, Herlin, Bastien, Ouvrard, Gaëlle, Bruneteau, Gaëlle, Hesters, Adèle, Gales, Ana Zenovia, Millet, Bruno, Lamari, Foudil, Lehericy, Stéphane, Navarro, Vincent, Rohaut, Benjamin, Demeret, Sophie, Maisonobe, Thierry, Yger, Marion, Degos, Bertrand, Mariani, Louise-Laure, Bouche, Christophe, Dzierzynski, Nathalie, Oquendo, Bruno, Ketz, Flora, Nguyen, An-Hung, Kas, Aurélie, Lubetzki, Catherine, Delattre, Jean-Yves, Corvol, Jean-Christophe, Fontaine, Bertrand, Thoumie, Philippe, Sharshar, Tarek, Alamowitch, Sonia, Apartis-Bourdieu, Emmanuelle, Peretti, Charles-Siegried, Ursu, Renata, Bourron, Kiyoka Kinugawa, Belmin, Joel, Pautas, Eric, Verny, Marc, Samson, Yves, Leder, Sara, Leger, Anne, Deltour, Sandrine, Baronnet, Flore, Touat, Mehdi, Sanson, Marc, Dehais, Caroline, Houillier, Caroline, Laigle-Donadey, Florence, Psimaras, Dimitri, Alenton, Agusti, Younan, Nadia, Villain, Nicolas, Del Mar Amador, Maria, Mezouar, Nicolas, Mangone, Graziella, Meneret, Aurelie, Hartmann, Andreas, Tarrano, Clement, Bendetowicz, David, Pradat, Pierre-François, Baulac, Michel, Pichit, Phintip, Chochon, Florence, Nguyen, An Hung, Porcher, Valerie, Demoule, Alexandre, Morawiec, Elise, Mayaux, Julien, Faure, Morgan, Ewenczyk, Claire, Coarelli, Giulia, Heinzmann, Anna, Masingue, Marion, Bassez, Guillaume, An, Isabelle, Worbe, Yulia, Lambrecq, Virginie, Debs, Rabab, Musat, Esteban Munoz, Lenglet, Timothee, Hanin, Aurelie, Chougar, Lydia, Shor, Nathalia, Pyatigorskaya, Nadya, Galanaud, Damien, Leclercq, Delphine, Cao, Albert, Marois, Clemence, Weiss, Nicolas, Le Guennec, Loic, Degos, Vincent, Jacquens, Alice, Similowski, Thomas, Morelot-Panzini, Capucine, Rotge, Jean-Yves, Saudreau, Bertrand, Sarni, Nassim, Girault, Nathalie, Leu, Smaranda, Thivard, Lionel, Mokhtari, Karima, Plu, Isabelle, Gonçalves, Bruno, Bottin, Laure, Haddad, Rebecca, 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, Gales, Ana, Brochard, Vanessa, Bourron, Kiyoka, Nguyen, An, Musat, Esteban, Malkinson, Tal, Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute (ICM), 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)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-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), Service de psychiatrie adulte [CHU Pitié-Salpêtière], CHU Pitié-Salpêtrière [AP-HP], Institut de la Mémoire et de la Maladie d'Alzheimer [Paris] (IM2A), Sorbonne Université (SU), Hôpital Rothschild [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de recherche en Myologie – U974 SU-INSERM, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Service des Pathologies du sommeil [CHU Pitié-Salpêtrière], Service de Biochimie Métabolique [CHU Pitié-Salpêtrière], Service de Neuroradiologie [CHU Pitié-Salpêtrière], Service de Neurophysiologie [CHU Pitié-Salpêtrière], Service des Soins Intensifs [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Médecine Gériatrique [CHU Pitié-Salpêtrière], Service d'Activités cliniques [CHU Pitié-Salpêtrière], Service de médecine nucléaire [CHU Pitié-Salpétrière], Service de neurologie 1 [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Centre de Recherche en Myologie, and Service de gériatrie [CHU Pitié-Salpêtrière]
Subjects: Pediatrics, medicine.medical_specialty, encephalitis, Encephalopathy, Context (language use), law.invention, 03 medical and health sciences, Cellular and Molecular Neuroscience, 0302 clinical medicine, law, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Intensive care, medicine, Critical illness polyneuropathy, Biological Psychiatry, 030304 developmental biology, 0303 health sciences, business.industry, AcademicSubjects/SCI01870, COVID-19, medicine.disease, encephalopathy, Comorbidity, Intensive care unit, 3. Good health, Psychiatry and Mental health, Neurology, Observational study, Original Article, AcademicSubjects/MED00310, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], business, critical illness neuropathy, 030217 neurology & neurosurgery, Encephalitis
Abstract: A variety of neuropsychiatric complications has been described in association with Covid-19 infection. Large scale studies presenting a wider picture of these complications and their relative frequency are lacking. The objective of our study was to describe the spectrum of neurological and psychiatric complications in patients with Covid-19 seen in a multidisciplinary hospital center over six months. We conducted a retrospective, observational study on all patients showing neurological or psychiatric symptoms in the context of Covid-19 seen in the medical and university neuroscience department of Assistance Publique Hopitaux de Paris -Sorbonne University. We collected demographic data, comorbidities, symptoms and severity of Covid-19 infection, neurological and psychiatric symptoms, neurological and psychiatric examination data and, when available, results from CSF analysis, MRI, EEG and EMG. A total of 249 Covid-19 patients with a de novo neurological or psychiatric manifestation were included in the database and 245 were included in the final analyses. One-hundred fourteen patients (47%) were admitted to the intensive care unit and 10 (4%) died. The most frequent neuropsychiatric complications diagnosed were encephalopathy (43%), critical illness polyneuropathy and myopathy (26%), isolated psychiatric disturbance (18%), and cerebrovascular disorders (16%). No patients showed CSF evidence of SARS-CoV-2. Encephalopathy was associated with older age and higher risk of death. Critical illness neuromyopathy was associated with an extended stay in the intensive care unit. The majority of these neuropsychiatric complications could be imputed to critical illness, intensive care and systemic inflammation, which contrasts with the paucity of more direct SARS-CoV-2-related complications or post-infection disorders., Graphical Abstract Graphical Abstract
Published: 2021

14. A phase II−III trial of olesoxime in subjects with amyotrophic lateral sclerosis

Author: Lenglet, T., Lacomblez, L., Abitbol, J. L., Ludolph, A., Mora, J. S., Robberecht, W., Shaw, P. J., Pruss, R. M., Cuvier, V., Meininger, V., Meininger, Vincent, Lacomblez, Pr Lucette, Salachas, François, Bruneteau, Gaëlle, Pradat, Pierre-François, Lenglet, Timothée, Lebouteux, Marie, Pibiri, Vanessa, Pouget, Jean, Verschueren, Annie, Campana-Salort, Emmanuelle, Attarian, Sharam, Couratier, Philippe, Nicolaud, Blerta, Lautrette, Géraldine, Camu, William, Morales, Raoul, Pageot, Nicolas, Desnuelle, Claude, Soriani, Marie-Hélène, Delmont, Emilien, Destee, Alain, Danel-Brunaud, Véronique, Devos, David, Vandenberghe, Nadia, Broussolle, Emmanuel, Vial, Christophe, Díaz, Rubén Martires Blanco, Bouhour, Françoise, Mora Pardina, Jesús S., Chaverri, Delia, Mascías, Javier, Hernández, María, Marín, Saul, Salas, Teresa, Moran, Yolanda, Robberecht, Wim, Van Damme, Philip, Demeestere, Jelle, Al-Chalabi, Ammar, Leigh, Nigel, Guevara, Carlos, Dimitrov, Nikolay, Wijesekera, Lokesh, Shaw, Pamela, McDermott, Christopher J., Rafiq, Muhammad, Ludolph, Albert, Dorst, Johannes, Waibel, Stefan, Weiland, Ulriche, Hendrich, Corinna, Dengler, Reinhardt, Petri, Suzanne, Kollewe, Katja Maureen, Schmalbach, Sonja, Cordes, Anna-Lena, Rath, Klaus, Bonzel, Linda, Bolat, Seza, Meyer, Thomas, Maier, André, Dullinger, Jörn, Linke, Peter, Holm, Teresa, Borisow, Nadja, Zierz, Stephan, and Hanisch, Frank
Published: 2014
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15. The wide spectrum of neuropsychiatric complications in Covid-19 patients within a multidisciplinary hospital context

Author: Delorme, Cécile, primary, Houot, Marion, additional, Rosso, Charlotte, additional, Carvalho, Stéphanie, additional, Nedelec, Thomas, additional, Maatoug, Redwan, additional, Pitron, Victor, additional, Gassama, Salimata, additional, Sambin, Sara, additional, Bombois, Stéphanie, additional, Herlin, Bastien, additional, Ouvrard, Gaëlle, additional, Bruneteau, Gaëlle, additional, Hesters, Adèle, additional, Gales, Ana Zenovia, additional, Millet, Bruno, additional, Lamari, Foudil, additional, Lehericy, Stéphane, additional, Navarro, Vincent, additional, Rohaut, Benjamin, additional, Demeret, Sophie, additional, Maisonobe, Thierry, additional, Yger, Marion, additional, Degos, Bertrand, additional, Mariani, Louise-Laure, additional, Bouche, Christophe, additional, Dzierzynski, Nathalie, additional, Oquendo, Bruno, additional, Ketz, Flora, additional, Nguyen, An-Hung, additional, Kas, Aurélie, additional, Delattre, Jean-Yves, additional, and Corvol, Jean-Christophe, additional
Published: 2020
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16. Muscle histone deacetylase 4 upregulation in amyotrophic lateral sclerosis: potential role in reinnervation ability and disease progression

Author: Bruneteau, Gaëlle, Simonet, Thomas, Bauché, Stéphanie, Mandjee, Nathalie, Malfatti, Edoardo, Girard, Emmanuelle, Tanguy, Marie-Laure, Behin, Anthony, Khiami, Frédéric, Sariali, Elhadi, Hell-Remy, Caroline, Salachas, François, Pradat, Pierre-François, Fournier, Emmanuel, Lacomblez, Lucette, Koenig, Jeanine, Romero, Norma Beatriz, Fontaine, Bertrand, Meininger, Vincent, Schaeffer, Laurent, and Hantaï, Daniel
Published: 2013
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17. Heterogeneous spectrum of neuropathies in Waldenströmʼs macroglobulinemia: a diagnostic strategy to optimize their management

Author: Viala, Karine, Stojkovic, Tanya, Doncker, Anne-Violaine, Maisonobe, Thierry, Lenglet, Timothée, Bruneteau, Gaëlle, Musset, Lucile, Neil, Jean, Léger, Jean-Marc, and Leblond, Véronique
Published: 2012
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18. Abnormal TDP-43 and FUS proteins in muscles of sporadic IBM: similarities in a TARDBP-linked ALS patient

Author: Hernandez Lain, Aurelio, Millecamps, Stéphanie, Dubourg, Odile, Salachas, François, Bruneteau, Gaëlle, Lacomblez, Lucette, LeGuern, Eric, Seilhean, Danielle, Duyckaerts, Charles, Meininger, Vincent, Mallet, Jacques, and Pradat, Pierre-François
Published: 2011
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19. SOD1, ANG, VAPB, TARDBP, and FUS mutations in familial amyotrophic lateral sclerosis: genotype–phenotype correlations

Author: Millecamps, Stéphanie, Salachas, François, Cazeneuve, Cécile, Gordon, Paul, Bricka, Bernard, Camuzat, Agnès, Guillot-Noël, Léna, Russaouen, Odile, Bruneteau, Gaëlle, Pradat, Pierre-François, Le Forestier, Nadine, Vandenberghe, Nadia, Danel-Brunaud, Véronique, 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
Published: 2010
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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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21. 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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22. NIV in amyotrophic lateral sclerosis: The ‘when’ and ‘how’ of the matter

Author: Morelot‐Panzini, Capucine, primary, Bruneteau, Gaëlle, additional, and Gonzalez‐Bermejo, Jesus, additional
Published: 2019
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23. Human diaphragm atrophy in amyotrophic lateral sclerosis is not predicted by routine respiratory measures

Author: Guimarães-Costa, Raquel, primary, Similowski, Thomas, additional, Rivals, Isabelle, additional, Morélot-Panzini, Capucine, additional, Nierat, Marie-Cécile, additional, Bui, Mai Thao, additional, Akbar, David, additional, Straus, Christian, additional, Romero, Norma Beatriz, additional, Michel, Patrick Pierre, additional, Menegaux, Fabrice, additional, Salachas, François, additional, Gonzalez-Bermejo, Jésus, additional, and Bruneteau, Gaëlle, additional
Published: 2019
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24. The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study

Author: Querin, Giorgia, primary, El Mendili, Mohamed-Mounir, additional, Lenglet, Timothée, additional, Behin, Anthony, additional, Stojkovic, Tanya, additional, Salachas, François, additional, Devos, David, additional, Le Forestier, Nadine, additional, del Mar Amador, Maria, additional, Debs, Rabab, additional, Lacomblez, Lucette, additional, Meininger, Vincent, additional, Bruneteau, Gaëlle, additional, Cohen-Adad, Julien, additional, Lehéricy, Stéphane, additional, Laforêt, Pascal, additional, Blancho, Sophie, additional, Benali, Habib, additional, Catala, Martin, additional, Li, Menghan, additional, Marchand-Pauvert, Véronique, additional, Hogrel, Jean-Yves, additional, Bede, Peter, additional, and Pradat, Pierre-François, additional
Published: 2019
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25. Emotional feeling in patients suffering from amyotrophic lateral sclerosis

Author: Unglik, Johanna, additional, Bungener, Catherine, additional, Delgadillo, Daniel, additional, Salachas, François, additional, Pradat, Pierre François, additional, Bruneteau, Gaëlle, additional, Lenglet, Timothée, additional, Le Forestier, Nadine, additional, Couratier, Philippe, additional, Vacher, Yannick, additional, and Lacomblez, Lucette, additional
Published: 2018
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26. No Benefit of Diaphragm Pacing in Upper Motor Neuron–Dominant Forms of Amyotrophic Lateral Sclerosis

Author: Morélot-Panzini, Capucine, primary, Nierat, Marie-Cécile, additional, Tanguy, Marie-Laure, additional, Bruneteau, Gaëlle, additional, Pradat, Pierre-François, additional, Salachas, François, additional, Gonzalez-Bermejo, Jésus, additional, and Similowski, Thomas, additional
Published: 2018
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27. 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. Specific Physical Exercise Improves Energetic Metabolism in the Skeletal Muscle of Amyotrophic-Lateral- Sclerosis Mice

Author: Desseille, Céline, primary, Deforges, Séverine, additional, Biondi, Olivier, additional, Houdebine, Léo, additional, D’amico, Domenico, additional, Lamazière, Antonin, additional, Caradeuc, Cédric, additional, Bertho, Gildas, additional, Bruneteau, Gaëlle, additional, Weill, Laure, additional, Bastin, Jean, additional, Djouadi, Fatima, additional, Salachas, François, additional, Lopes, Philippe, additional, Chanoine, Christophe, additional, Massaad, Charbel, additional, and Charbonnier, Frédéric, additional
Published: 2017
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29. Early diaphragm pacing in patients with amyotrophic lateral sclerosis (RespiStimALS): a randomised controlled triple-blind trial

Author: Gonzalez-Bermejo, Jésus, primary, Morélot-Panzini, Capucine, additional, Tanguy, Marie-Laure, additional, Meininger, Vincent, additional, Pradat, Pierre-François, additional, Lenglet, Timothée, additional, Bruneteau, Gaëlle, additional, Forestier, Nadine Le, additional, Couratier, Philippe, additional, Guy, Nathalie, additional, Desnuelle, Claude, additional, Prigent, Hélène, additional, Perrin, Christophe, additional, Attali, Valérie, additional, Fargeot, Catherine, additional, Nierat, Marie-Cécile, additional, Royer, Catherine, additional, Ménégaux, Fabrice, additional, Salachas, François, additional, and Similowski, Thomas, additional
Published: 2016
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30. Émotions ressenties chez des patients atteints de sclérose latérale amyotrophique.

Author: Unglik, Johanna, Bungener, Catherine, Delgadillo, Daniel, Salachas, François, Pradat, Pierre François, Bruneteau, Gaëlle, Lenglet, Timothée, Le Forestier, Nadine, Couratier, Philippe, Vacher, Yannick, and Lacomblez, Lucette
Abstract: Copyright of Gériatrie et Psychologie Neuropsychiatrie du Vieillissement is the property of John Libbey Eurotext Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
Published: 2018
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31. Etude de la jonction neuromusculaire dans la sclérose latérale amyotrophique

Author: Bruneteau, Gaëlle, Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière (CRICM), Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), Université Pierre et Marie Curie - Paris VI, Daniel Hantaï, 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), and STAR, ABES
Subjects: HDAC, Innervation, Jonction neuromusculaire, Cellule de Schwann terminale, Sclérose latérale amyotrophique, Neuromuscular junction, Transmission neuromusculaire, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], [SDV.NEU] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], Amyotrophic lateral sclerosis
Abstract: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disorder affecting motor neurons, usually leading to death in 3 to 5 years. The only treatment currently available, riluzole, has a modest effect on survival. Functional alterations of the neuromuscular junction (NMJ) have been reported in ALS, but their pathophysiological significance remains unknown. We studied the morphology of neuromuscular junctions in muscle samples collected from 11 ALS patients, using confocal and electron microscopy. Functional analysis of the NMJs was performed using surface-recording of compound motor action potentials after repetitive nerve stimulation at slow stimulus rate. A significant decrement (>10%), suggesting impairment of the neuromuscular transmission, was present in 45% of the patients. Morphological alterations of the NMJs were present in all ALS patients even at the early-stages. Beside denervation-induced morphological changes, one third of the NMJs showed abnormal spike-like areas of the outer edge of the postsynaptic primary gutter. A marked interposition of the terminal Schwann cell between the nerve terminal and the postsynaptic membrane, which was likely to alter synaptic transmission, was sometimes present. We found a significantly greater compensatory reinnervation in muscle from patients with slowly progressive ALS. Furthermore, we identified that the muscle molecular factor histone deacetylase 4 could play a key role in muscle reinnervation and disease progression in patients with ALS. This work has highlighted the presence of major morphological changes at the NMJs of ALS patients and identified potential new targets for future treatment., La Sclérose Latérale Amyotrophique (SLA) est une affection neurodégénérative touchant les motoneurones, habituellement mortelle en 3 à 5 ans. La cause de la maladie n'est pas connue et le seul traitement actuellement disponible ne permet qu'un allongement modeste de la survie. Des altérations fonctionnelles de la jonction neuromusculaire (JNM) ont été rapportées dans la SLA mais leur origine physiopathologique n'est pas connue. Nous avons étudié les JNM chez 11 patients atteints de SLA, en associant étude morphologique en microscopie confocale et analyse ultrastructurale. L'analyse fonctionnelle réalisée en EMG de surface retrouvait une anomalie de transmission neuromusculaire (décrément > 10%) chez 45% des patients. Des altérations morphologiques des JNM étaient visibles chez tous les patients, y compris au stade précoce de la maladie. Associé aux anomalies en rapport avec le phénomène de dénervation, nous avons observé un aspect anormal de spiculation de la gouttière primaire dans environ un tiers des cas. Une interposition marquée de la cellule de Schwann terminale entre la terminaison nerveuse et la membrane postsynaptique, pouvant altérer la transmission synaptique, était parfois visible. Nous avons objectivé une réinnervation compensatrice significativement plus importante chez les patients présentant une SLA d'évolution lente et montré que certains facteurs moléculaires musculaires comme l'histone déacétylase 4 pourraient jouer un rôle crucial dans la capacité de réinnervation. Ce travail a mis en évidence des altérations morphologiques majeures au niveau des JNM des patients atteints de SLA et a permis d'identifier des cibles thérapeutiques potentielles.
Published: 2014

32. A study of neuromuscular junction in amyotrophic lateral sclerosis

Author: Bruneteau, Gaëlle, STAR, ABES, Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière (CRICM), 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 VI, and Daniel Hantaï
Subjects: HDAC, Jonction neuromusculaire, Innervation, Sclérose latérale amyotrophique, Cellule de Schwann terminale, Neuromuscular junction, Transmission neuromusculaire, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], [SDV.NEU] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], Amyotrophic lateral sclerosis
Abstract: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disorder affecting motor neurons, usually leading to death in 3 to 5 years. The only treatment currently available, riluzole, has a modest effect on survival. Functional alterations of the neuromuscular junction (NMJ) have been reported in ALS, but their pathophysiological significance remains unknown. We studied the morphology of neuromuscular junctions in muscle samples collected from 11 ALS patients, using confocal and electron microscopy. Functional analysis of the NMJs was performed using surface-recording of compound motor action potentials after repetitive nerve stimulation at slow stimulus rate. A significant decrement (>10%), suggesting impairment of the neuromuscular transmission, was present in 45% of the patients. Morphological alterations of the NMJs were present in all ALS patients even at the early-stages. Beside denervation-induced morphological changes, one third of the NMJs showed abnormal spike-like areas of the outer edge of the postsynaptic primary gutter. A marked interposition of the terminal Schwann cell between the nerve terminal and the postsynaptic membrane, which was likely to alter synaptic transmission, was sometimes present. We found a significantly greater compensatory reinnervation in muscle from patients with slowly progressive ALS. Furthermore, we identified that the muscle molecular factor histone deacetylase 4 could play a key role in muscle reinnervation and disease progression in patients with ALS. This work has highlighted the presence of major morphological changes at the NMJs of ALS patients and identified potential new targets for future treatment., La Sclérose Latérale Amyotrophique (SLA) est une affection neurodégénérative touchant les motoneurones, habituellement mortelle en 3 à 5 ans. La cause de la maladie n'est pas connue et le seul traitement actuellement disponible ne permet qu'un allongement modeste de la survie. Des altérations fonctionnelles de la jonction neuromusculaire (JNM) ont été rapportées dans la SLA mais leur origine physiopathologique n'est pas connue. Nous avons étudié les JNM chez 11 patients atteints de SLA, en associant étude morphologique en microscopie confocale et analyse ultrastructurale. L'analyse fonctionnelle réalisée en EMG de surface retrouvait une anomalie de transmission neuromusculaire (décrément > 10%) chez 45% des patients. Des altérations morphologiques des JNM étaient visibles chez tous les patients, y compris au stade précoce de la maladie. Associé aux anomalies en rapport avec le phénomène de dénervation, nous avons observé un aspect anormal de spiculation de la gouttière primaire dans environ un tiers des cas. Une interposition marquée de la cellule de Schwann terminale entre la terminaison nerveuse et la membrane postsynaptique, pouvant altérer la transmission synaptique, était parfois visible. Nous avons objectivé une réinnervation compensatrice significativement plus importante chez les patients présentant une SLA d'évolution lente et montré que certains facteurs moléculaires musculaires comme l'histone déacétylase 4 pourraient jouer un rôle crucial dans la capacité de réinnervation. Ce travail a mis en évidence des altérations morphologiques majeures au niveau des JNM des patients atteints de SLA et a permis d'identifier des cibles thérapeutiques potentielles.
Published: 2014

33. Cat Bite: An Unusual Cause of Foot Drop

Author: Amador, Maria del Mar, primary, Bruneteau, Gaëlle, additional, and Degos, Bertrand, additional
Published: 2016
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34. Endplate denervation correlates with Nogo-A muscle expression in amyotrophic lateral sclerosis patients

Author: Bruneteau, Gaëlle, primary, Bauché, Stéphanie, additional, Gonzalez de Aguilar, Jose Luis, additional, Brochier, Guy, additional, Mandjee, Nathalie, additional, Tanguy, Marie-Laure, additional, Hussain, Ghulam, additional, Behin, Anthony, additional, Khiami, Frédéric, additional, Sariali, Elhadi, additional, Hell-Remy, Caroline, additional, Salachas, François, additional, Pradat, Pierre-François, additional, Lacomblez, Lucette, additional, Nicole, Sophie, additional, Fontaine, Bertrand, additional, Fardeau, Michel, additional, Loeffler, Jean-Philippe, additional, Meininger, Vincent, additional, Fournier, Emmanuel, additional, Koenig, Jeanine, additional, and Hantaï, Daniel, additional
Published: 2015
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35. Fréquence et caractérisation des anomalies de transmission neuromusculaire chez les patients atteints de Sclérose Latérale Amyotrophique

Author: Bruneteau, Gaelle, Guimarães-Costa, Raquel, Salachas, François, Pradat, Pierre-François, Lacomblez, Lucette, Meininger, Vincent, and Fournier, Emmanuel
Published: 2017
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36. Plasma Peptide Biomarker Discovery for Amyotrophic Lateral Sclerosis by MALDI –TOF Mass Spectrometry Profiling

Author: Conraux, Laurence, primary, Pech, Catherine, additional, Guerraoui, Halim, additional, Loyaux, Denis, additional, Ferrara, Pascual, additional, Guillemot, Jean-Claude, additional, Meininger, Vincent, additional, Pradat, Pierre-François, additional, Salachas, François, additional, Bruneteau, Gaëlle, additional, Le Forestier, Nadine, additional, and Lacomblez, Lucette, additional
Published: 2013
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37. Cannabis for the treatment of Amyotrophic Lateral Sclerosis: what is the patients’ view?

Author: Lacroix, Clémence, Guilhaumou, Romain, Micallef, Joëlle, Bruneteau, Gaëlle, Desnuelle, Claude, and Blin, Olivier
Abstract: Cannabis may have therapeutic benefits to relieve symptoms of amyotrophic lateral sclerosis (ALS) thanks to its pleiotropic pharmacological activity. This study is the first to present a large questionnaire-based survey about the “real-life” situation regarding cannabis use in the medical context in ALS patients in France. There were 129 respondents and 28 reported the use of cannabis (21.7%) to relieve symptoms of ALS. Participants mostly reported the use of cannabidiol (CBD) oil and cannabis weed and declared benefits both on motor (rigidity, cramps, fasciculations) and non-motor (sleep quality, pain, emotional state, quality of life, depression) symptoms and only eight reported minor adverse reactions (drowsiness, euphoria and dry mouth). Even if cannabis is mostly used outside medical pathways and could expose patients to complications (street and uncontrolled drugs, drug-drug interactions, adverse effects…), most of the participants reported “rational” consumption (legal cannabinoids, with only few combustion and adverse reactions). Despite some limitations, this study highlights the need for further research on the potential benefits of cannabis use for the management of ALS motor and non-motor symptoms. Indeed, there is an urgent need and call for and from patients to know more about cannabis and secure its use in a medical context.
Published: 2023
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38. Diaphragm pacing improves sleep in patients with amyotrophic lateral sclerosis

Author: Gonzalez-Bermejo, Jesus, primary, Morélot-Panzini, Capucine, additional, Salachas, François, additional, Redolfi, Stefania, additional, Straus, Christian, additional, Becquemin, Marie-Hélène, additional, Arnulf, Isabelle, additional, Pradat, Pierre-François, additional, Bruneteau, Gaëlle, additional, Ignagni, Anthony R., additional, Diop, Moustapha, additional, Onders, Raymond, additional, Nelson, Teresa, additional, Menegaux, Fabrice, additional, Meininger, Vincent, additional, and Similowski, Thomas, additional
Published: 2011
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39. Muscle Gene Expression Is a Marker of Amyotrophic Lateral Sclerosis Severity

Author: Pradat, Pierre-François, primary, Dubourg, Odile, additional, de Tapia, Marc, additional, di Scala, Franck, additional, Dupuis, Luc, additional, Lenglet, Timothee, additional, Bruneteau, Gaëlle, additional, Salachas, François, additional, Lacomblez, Lucette, additional, Corvol, Jean-Christophe, additional, Demougin, Philippe, additional, Primig, Michael, additional, Meininger, Vincent, additional, Loeffler, Jean-Philippe, additional, and Gonzalez de Aguilar, Jose-Luis, additional
Published: 2011
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40. Platelet Serotonin Level Predicts Survival in Amyotrophic Lateral Sclerosis

Author: Dupuis, Luc, primary, Spreux-Varoquaux, Odile, additional, Bensimon, Gilbert, additional, Jullien, Philippe, additional, Lacomblez, Lucette, additional, Salachas, François, additional, Bruneteau, Gaëlle, additional, Pradat, Pierre-François, additional, Loeffler, Jean-Philippe, additional, and Meininger, Vincent, additional
Published: 2010
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41. Diagnostic d’une sclérose latérale amyotrophique chez un patient traité par anti-TNFα pour spondylarthrite ankylosante : association fortuite ou nouvel effet secondaire des anti-TNFα ?

Author: Loustau, Valentine, primary, Foltz, Violaine, additional, Poulain, Cécile, additional, Rozenberg, Sylvie, additional, and Bruneteau, Gaëlle, additional
Published: 2009
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42. Diagnosis of amyotrophic lateral sclerosis in a patient treated with TNFα blockers for ankylosing spondylitis: Fortuitus association or new side effect of TNFα blockers?

Author: Loustau, Valentine, primary, Foltz, Violaine, additional, Poulain, Cécile, additional, Rozenberg, Sylvie, additional, and Bruneteau, Gaëlle, additional
Published: 2009
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43. Causes of death in a post‐mortem series of ALS patients

Author: Corcia, Philippe, primary, Pradat, Pierre‐François, additional, Salachas, François, additional, Bruneteau, Gaëlle, additional, le Forestier, Nadine, additional, Seilhean, Danielle, additional, Hauw, Jean‐Jacques, additional, and Meininger, Vincent, additional
Published: 2008
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44. Assessing Perfusion and Capillary Permeability Changes Induced by a VEGF Inhibitor in Human Tumor Xenografts using Macromolecular MR Imaging Contrast Media

Author: Clément, Olivier, primary, Pradel, Clément, additional, Siauve, Nathalie, additional, Frouin, Frédérique, additional, Bruneteau, Gaëlle, additional, Kahn, Edmond, additional, Frija, Guy, additional, and Cuénod, Charles-André, additional
Published: 2002
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45. Diaphragm pacing improves sleep in patients with amyotrophic lateral sclerosis.

Author: Gonzalez-Bermejo, Jesus, Morélot-Panzini, Capucine, Salachas, François, Redolfi, Stefania, Straus, Christian, Becquemin, Marie-Hélène, Arnulf, Isabelle, Pradat, Pierre-François, Bruneteau, Gaëlle, Ignagni, Anthony R., Diop, Moustapha, Onders, Raymond, Nelson, Teresa, Menegaux, Fabrice, Meininger, Vincent, and Similowski, Thomas
Subjects: AMYOTROPHIC lateral sclerosis, DIAPHRAGM (Anatomy), RESPIRATORY insufficiency, ELECTRIC stimulation, SLEEP, HEALTH, PATIENTS
Abstract: In amyotrophic lateral sclerosis (ALS) patients, respiratory insufficiency is a major burden. Diaphragm conditioning by electrical stimulation could interfere with lung function decline by promoting the development of type 1 muscle fibres. We describe an ancillary study to a prospective, non-randomized trial (NCT00420719) assessing the effects of diaphragm pacing on forced vital capacity (FVC). Sleep-related disturbances being early clues to diaphragmatic dysfunction, we postulated that they would provide a sensitive marker. Stimulators were implanted laparoscopically in the diaphragm close to the phrenic motor point in 18 ALS patients for daily conditioning. ALS functioning score (ALSFRS), FVC, sniff nasal inspiratory pressure (SNIP), and polysomnographic recordings (PSG, performed with the stimulator turned off) were assessed before implantation and after four months of conditioning ( n == 14). Sleep efficiency improved (69 ± 15% to 75 ± 11%, p == 0.0394) with fewer arousals and micro-arousals. This occurred against a background of deterioration as ALSFRS-R, FVC, and SNIP declined. There was, however, no change in NIV status or the ALSFRS respiratory subscore, and the FVC decline was mostly due to impaired expiration. Supporting a better diaphragm function, apnoeas and hypopnoeas during REM sleep decreased. In conclusion, in these severe patients not expected to experience spontaneous improvements, diaphragm conditioning improved sleep and there were hints at diaphragm function changes. [ABSTRACT FROM AUTHOR]
Published: 2012
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46. Muscle gene expression is a marker of amyotrophic lateral sclerosis severity.

Author: Pradat PF, Dubourg O, de Tapia M, di Scala F, Dupuis L, Lenglet T, Bruneteau G, Salachas F, Lacomblez L, Corvol JC, Demougin P, Primig M, Meininger V, Loeffler JP, and Gonzalez de Aguilar JL
Subjects: Adolescent, Adult, Aged, Amyotrophic Lateral Sclerosis metabolism, Amyotrophic Lateral Sclerosis pathology, Case-Control Studies, Deltoid Muscle pathology, Female, Genetic Markers, Humans, Male, Middle Aged, Muscular Atrophy, Amyotrophic Lateral Sclerosis genetics, Deltoid Muscle metabolism, Gene Expression Profiling, Gene Expression Regulation
Abstract: Background: Amyotrophic lateral sclerosis (ALS) is a fatal adult-onset degenerative disease characterized by the loss of upper and lower motor neurons leading to progressive muscle atrophy and paralysis. The lack of molecular markers of the progression of disease is detrimental to clinical practice and therapeutic trials., Objective: This study was designed to identify gene expression changes in skeletal muscle that could reliably define the degree of disease severity., Methods: Gene expression profiles were obtained from the deltoid muscles of ALS patients and healthy subjects. Changes in differentially expressed genes were compared to the status of deltoid muscle disability, as determined by manual muscle testing, electrophysiology and the degree of myofiber atrophy. Functionally related genes were grouped by annotation analysis, and deltoid muscle injury was predicted using binary tree classifiers., Results: Two sets of 25 and 70 transcripts appeared differentially regulated exclusively in early and advanced states of deltoid muscle impairment, respectively. The expression of another set of 198 transcripts correlated with a composite score of muscle injury combining manual muscle testing and histological examination. From the totality of these expression changes, 155 transcripts distinguished advanced from early deltoid muscle impairment with 80% sensitivity and 100% specificity. Nine of these transcripts, known also to be regulated in ALS mouse and surgically denervated muscle, predicted the advanced disease status with 100% sensitivity and specificity., Conclusion: We provide robust gene expression changes that can be of practical use when monitoring ALS status and the effects of disease-modifying drugs., (Copyright © 2011 S. Karger AG, Basel.)
Published: 2012
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47. Questioning on the role of D amino acid oxidase in familial amyotrophic lateral sclerosis.

Author: Millecamps S, Da Barroca S, Cazeneuve C, Salachas F, Pradat PF, Danel-Brunaud V, Vandenberghe N, Lacomblez L, Le Forestier N, Bruneteau G, Camu W, Brice A, Meininger V, and LeGuern E
Subjects: Amino Acid Sequence, Amino Acid Substitution, Base Sequence, Conserved Sequence, DNA genetics, DNA Mutational Analysis, Exons, Female, Heterozygote, Humans, Male, Molecular Sequence Data, Mutation, Missense, Pedigree, Polymorphism, Single Nucleotide, Sequence Homology, Amino Acid, Species Specificity, Amyotrophic Lateral Sclerosis enzymology, Amyotrophic Lateral Sclerosis genetics, D-Amino-Acid Oxidase genetics, Genetic Variation
Published: 2010
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48. [SLA: a long way since Charcot].

Author: Pradat PF, Bruneteau G, Salachas F, and Meininger V
Subjects: Apoptosis, Environment, Humans, Amyotrophic Lateral Sclerosis etiology, Amyotrophic Lateral Sclerosis pathology
Published: 2003

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