Search

Your search keyword '"Marie Delliaux"' showing total 31 results
Start Over Author "Marie Delliaux"
31 results on '"Marie Delliaux"'

1. Are Upper-Body Axial Symptoms a Feature of Early Parkinson's Disease?

Author

Caroline Moreau, David Devos, Guillaume Baille, Arnaud Delval, Céline Tard, Thierry Perez, Nicolas Danel-Buhl, David Seguy, Julien Labreuche, Alain Duhamel, Marie Delliaux, Kathy Dujardin, and Luc Defebvre

Subjects
Medicine, Science
Abstract

BACKGROUND:Axial disorders are considered to appear late in the course of Parkinson's disease (PD). The associated impact on quality of life (QoL) and survival and the lack of an effective treatment mean that understanding and treating axial disorders is a key challenge. However, upper-body axial disorders (namely dysarthria, swallowing and breathing disorders) have never been prospectively assessed in early-stage PD patients. OBJECTIVES:To characterize upper-body axial symptoms and QoL in consecutive patients with early-stage PD. METHODS:We prospectively enrolled 66 consecutive patients with early-stage PD (less than 3 years of disease progression) and assessed dysarthria, dysphagia and respiratory function (relative to 36 controls) using both objective and patient-reported outcomes. RESULTS:The mean disease duration was 1.26 years and the mean UPDRS motor score was 19.4 out of 108. 74% of the patients presented slight dysarthria (primarily dysprosodia). Men appeared to be more severely affected (i.e. dysphonia). This dysfunction was strongly correlated with low swallowing speed (despite the absence of complaints about dysphagia), respiratory insufficiency and poor QoL. Videofluorography showed that oral-phase swallowing disorders affected 60% of the 31 tested patients and that pharyngeal-phase disorders affected 21%. 24% of the patients reported occasional dyspnea, which was correlated with anxiety in women but not in men. Marked diaphragmatic dysfunction was suspected in 42% of the patients (predominantly in men). CONCLUSION:Upper body axial symptoms were frequent in men with early-stage PD, whereas women presented worst non-motor impairments. New assessment methods are required because currently available tools do not reliably detect these upper-body axial disorders.

Published
2016
Full Text
View/download PDF

2. Expanding the phenotype of SCA19/22: Parkinsonism, cognitive impairment and epilepsy

Author

Jean-Christophe Cuvellier, Marie Delliaux, Kathy Dujardin, Delphine Dellacherie, Bernard Sablonnière, Isabelle Strubi-Vuillaume, David Devos, Luc Defebvre, Vincent Huin, Audrey Riquet, Marine Brion, Caroline Moreau, Jean-Marie Cuisset, HUIN, Vincent, Centre de Recherche Jean-Pierre AUBERT Neurosciences et Cancer - U837 (JPArc), Université Lille Nord de France (COMUE)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, Pôle de Biologie Pathologie Génétique [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Troubles cognitifs dégénératifs et vasculaires - U 1171 (TCDV), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Neurologie et Pathologies du Mouvement [CHU Lille], Service de neuropédiatrie [CHU Lille], Troubles cognitifs dégénératifs et vasculaires - U 1171 - EA 1046 (TCDV), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre de Recherche Jean-Pierre AUBERT Neurosciences et Cancer - U1172 Inserm - U837 (JPArc), Centre National de la Recherche Scientifique (CNRS)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Université Lille Nord de France (COMUE)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de Biologie Pathologie et Génétique, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Institut d'Immunologie [CHRU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Pôle de Biologie Pathologie Génétique [CHU Lille], and Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Lille Nord de France (COMUE)-Université de Lille

Subjects
Male, 0301 basic medicine, Pediatrics, Parkinson's disease, [SDV]Life Sciences [q-bio], [SDV.GEN] Life Sciences [q-bio]/Genetics, Mesh:Adult, [SDV.BBM.BM] Life Sciences [q-bio]/Biochemistry, Molecular Biology/Molecular biology, [SCCO]Cognitive science, Epilepsy, Mesh:Adolescent, 0302 clinical medicine, Medicine, Child, Neurogenetics, Mesh:Epilepsy/genetics, Mesh:Aged, Spinocerebellar Degenerations, Aged, 80 and over, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, medicine.diagnostic_test, Parkinsonism, Mesh:Spinocerebellar Degenerations/complications, Cognition, Middle Aged, Mesh:Cognitive Dysfunction/genetics, Pedigree, 3. Good health, Phenotype, Neurology, Spinocerebellar ataxia, [SDV.BBM.GTP] Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], Female, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], medicine.symptom, Adult, Mesh:Female, medicine.medical_specialty, Adolescent, Cognitive disorders, Mesh:Child, Mesh:Male, [SDV.GEN.GH] Life Sciences [q-bio]/Genetics/Human genetics, 03 medical and health sciences, Parkinsonian Disorders, Mesh:Middle Aged, [SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], Mesh:80 and over, Humans, Cognitive Dysfunction, Quantitative Biology - Genomics, [SDV.NEU] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], Aged, Genetic testing, Genomics (q-bio.GN), [SDV.GEN]Life Sciences [q-bio]/Genetics, Cerebellar ataxia, business.industry, Mesh:Pedigree, Mesh:Phenotype, [SDV.BBM.BM]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Molecular biology, [SCCO] Cognitive science, medicine.disease, Neuropsychiatric disorder, Mesh:Humans, 030104 developmental biology, KCND3 mutation, [SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics, Mesh:Parkinsonian Disorders/genetics, Parkinson''s disease, FOS: Biological sciences, Neurology (clinical), Geriatrics and Gerontology, business, Neuroscience, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, 030217 neurology & neurosurgery
Abstract

International audience; BACKGROUND: Spinocerebellar ataxia types 19 and 22 (SCA19/22) are rare conditions in which relatively isolated cerebellar involvement is frequently associated with cognitive impairment. Here, we report on new clinical features and provide details of the cognitive profile in two SCA19/22 families.METHODS: Two families displaying an autosomal-dominant form of cerebellar ataxia underwent clinical examinations and genetic testing.RESULTS: In addition to the classical clinical features of SCA, a wide spectrum of cognitive disorders (including visuospatial impairments) was observed. Eight patients had mild Parkinsonism, and five had epilepsy. Genetic testing showed that the KCND3 mutation (c.679_681delTTC, p.F227del) was present in both families.CONCLUSIONS: Our findings broaden the phenotypic spectrum of SCA19/22, and suggest that KCND3 should be included in the list of candidate genes for epilepsy, Parkinsonism and cognitive impairment.

Published
2017
Full Text
View/download PDF

3. Identification of genetic variants associated with Huntington's disease progression

Author

Davina J Hensman Moss, Antonio F Pardiñas, Douglas Langbehn, Kitty Lo, Blair R Leavitt, Raymund Roos, Alexandra Durr, Simon Mead, Peter Holmans, Lesley Jones, Sarah J Tabrizi, A Coleman, R Dar Santos, J Decolongon, A Sturrock, E Bardinet, C Jauff Ret, D Justo, S Lehericy, C Marelli, K Nigaud, R Valabrègue, SJA van den Bogaard, E M Dumas, J van der Grond, EP t'Hart, C Jurgens, M-N Witjes-Ane, N Arran, J Callaghan, C Stopford, C Frost, R Jones, N Hobbs, N Lahiri, R Ordidge, G Owen, T Pepple, J Read, M Say, E Wild, A Patel, N C Fox, C Gibbard, I Malone, H Crawford, D Whitehead, S Keenan, D M Cash, C Berna, N Bechtel, S Bohlen, A Hoff Man, P Kraus, E Axelson, C Wang, T Acharya, S Lee, W Monaco, C Campbell, S Queller, K Whitlock, M Campbell, E Frajman, C Milchman, A O'Regan, I Labuschagne, J Stout, B Landwehrmeyer, D Craufurd, R Scahill, S Hicks, C Kennard, H Johnson, A Tobin, HD Rosas, R Reilmann, B Borowsky, C Pourchot, S C Andrews, Anne-Catherine Bachoud-Lévi, Anna Rita Bentivoglio, Ida Biunno, Raphael Bonelli, Jean-Marc Burgunder, Stephen Dunnett, Joaquim Ferreira, Olivia Handley, Arvid Heiberg, Torsten Illmann, G. Bernhard Landwehrmeyer, Jamie Levey, Maria A. Ramos-Arroyo, Jørgen Nielsen, Susana Pro Koivisto, Markku Päivärinta, Raymund A.C. Roos, A Rojo Sebastián, Sarah Tabrizi, Wim Vandenberghe, Christine Verellen-Dumoulin, Tereza Uhrova, Jan Wahlström, Jacek Zaremba, Verena Baake, Katrin Barth, Monica Bascuñana Garde, Sabrina Betz, Reineke Bos, Jenny Callaghan, Adrien Come, Leonor Correia Guedes, Daniel Ecker, Ana Maria Finisterra, Ruth Fullam, Mette Gilling, Lena Gustafsson, Olivia J Handley, Carina Hvalstedt, Christine Held, Kerstin Koppers, Claudia Lamanna, Matilde Laurà, Asunción Martínez Descals, Saül Martinez-Horta, Tiago Mestre, Sara Minster, Daniela Monza, Lisanne Mütze, Martin Oehmen, Michael Orth, Hélène Padieu, Laurent Paterski, Nadia Peppa, Martina Di Renzo, Amandine Rialland, Niini Røren, Pavla Šašinková, Erika Timewell, Jenny Townhill, Patricia Trigo Cubillo, Wildson Vieira da Silva, Marleen R van Walsem, Carina Whalstedt, Marie-Noelle Witjes-Ané, Grzegorz Witkowski, Abigail Wright, Daniel Zielonka, Eugeniusz Zielonka, Paola Zinzi, Raphael M. Bonelli, Sabine Lilek, Karen Hecht, Brigitte Herranhof, Anna Holl, Hans-Peter Kapfhammer, Michael Koppitz, Markus Magnet, Nicole Müller, Daniela Otti, Annamaria Painold, Karin Reisinger, Monika Scheibl, Helmut Schöggl, Jasmin Ullah, Eva-Maria Braunwarth, Florian Brugger, Lisa Buratti, Eva-Maria Hametner, Caroline Hepperger, Christiane Holas, Anna Hotter, Anna Hussl, Christoph Müller, Werner Poewe, Klaus Seppi, Fabienne Sprenger, Gregor Wenning, Andrea Boogaerts, Godelinde Calmeyn, Isabelle Delvaux, Dirk Liessens, Nele Somers, Michel Dupuit, Cécile Minet, Dominique van Paemel, Pascale Ribaï, Dimphna van Reijen, Jirí Klempír, Veronika Majerová, Jan Roth, Irena Stárková, Lena E. Hjermind, Oda Jacobsen, Jørgen E. Nielsen, Ida Unmack Larsen, Tua Vinther-Jensen, Heli Hiivola, Hannele Hyppönen, Kirsti Martikainen, Katri Tuuha, Philippe Allain, Dominique Bonneau, Marie Bost, Bénédicte Gohier, Marie-Anne Guérid, Audrey Olivier, Adriana Prundean, Clarisse Scherer-Gagou, Christophe Verny, Blandine Babiloni, Sabrina Debruxelles, Charlotte Duché, Cyril Goizet, Laetitia Jameau, Danielle Lafoucrière, Umberto Spampinato, Rekha Barthélémy, Christelle De Bruycker, Maryline Cabaret Anne-Sophie Carette, Eric Decorte Luc Defebvre, Marie Delliaux, Arnaud Delval, Alain Destee, Kathy Dujardin, Marie-Hélène Lemaire, Sylvie Manouvrier, Mireille Peter, Lucie Plomhouse, Bernard Sablonnière, Clémence Simonin, Stéphanie Thibault-Tanchou, Isabelle Vuillaume, Marcellin Bellonet, Hassan Berrissoul, Stéphanie Blin, Françoise Courtin, Cécile Duru, Véronique Fasquel, Olivier Godefroy, Pierre Krystkowiak, Béatrice Mantaux, Martine Roussel, Sandrine Wannepain, Jean-Philippe Azulay, Marie Delfini, Alexandre Eusebio, Frédérique Fluchere, Laura Mundler, Mathieu Anheim, Celine Julié, Ouhaid Lagha Boukbiza, Nadine Longato, Gabrielle Rudolf, Christine Tranchant, Marie-Agathe Zimmermann, Christoph Michael Kosinski, Eva Milkereit, Daniela Probst, Kathrin Reetz, Christian Sass, Johannes Schiefer, Christiane Schlangen, Cornelius J. Werner, Harald Gelderblom, Josef Priller, Harald Prüß, Eike Jakob Spruth, Gisa Ellrichmann, Lennard Herrmann, Rainer Hoffmann, Barbara Kaminski, Peter Kotz, Christian Prehn, Carsten Saft, Herwig Lange, Robert Maiwald, Matthias Löhle, Antonia Maass, Simone Schmidt, Cecile Bosredon, Alexander Storch, Annett Wolz, Martin Wolz, Philipp Capetian, Johann Lambeck, Birgit Zucker, Kai Boelmans, Christos Ganos, Walburgis Heinicke, Ute Hidding, Jan Lewerenz, Alexander Münchau, Jenny Schmalfeld, Lars Stubbe, Simone Zittel, Gabriele Diercks, Dirk Dressler, Heike Gorzolla, Christoph Schrader, Pawel Tacik, Michael Ribbat, Bernhard Longinus, Katrin Bürk, Jens Carsten Möller, Ida Rissling, Mark Mühlau, Alexander Peinemann, Michael Städtler, Adolf Weindl, Juliane Winkelmann, Cornelia Ziegler, Natalie Bechtel, Heike Beckmann, Stefan Bohlen, Eva Hölzner, Ralf Reilmann, Stefanie Rohm, Silke Rumpf, Sigrun Schepers, Natalia Weber, Matthias Dose, Gabriele Leythäuser, Ralf Marquard, Tina Raab, Alexandra Wiedemann, Andrea Buck, Julia Connemann, Carolin Geitner, Andrea Kesse, Bernhard Landwehrmeyer, Christina Lang, Franziska Lezius, Solveig Nepper, Anke Niess, Ariane Schneider, Daniela Schwenk, Sigurd Süßmuth, Sonja Trautmann, Patrick Weydt, Claudia Cormio, Vittorio Sciruicchio, Claudia Serpino, Marina de Tommaso, Sabina Capellari, Pietro Cortelli, Roberto Galassi, Giovanni Rizzo, Roberto Poda, Cesa Scaglione, Elisabetta Bertini, Elena Ghelli, Andrea Ginestroni, Francesca Massaro, Claudia Mechi, Marco Paganini, Silvia Piacentini, Silvia Pradella, Anna Maria Romoli, Sandro Sorbi, Giovanni Abbruzzese, Monica Bandettini di Poggio, Giovanna Ferrandes, Paola Mandich, Roberta Marchese, Alberto Albanese, Daniela Di Bella, Anna Castaldo, Stefano Di Donato, Cinzia Gellera, Silvia Genitrini, Caterina Mariotti, Lorenzo Nanetti, Dominga Paridi, Paola Soliveri, Chiara Tomasello, Giuseppe De Michele, Luigi Di Maio, Marco Massarelli, Silvio Peluso, Alessandro Roca, Cinzia Valeria Russo, Elena Salvatore, Pierpaolo Sorrentino, Enrico Amico, Mariagrazia Favellato, Annamaria Griguoli, Irene Mazzante, Martina Petrollini, Ferdinando Squitieri, Barbara D'Alessio, Chiara Esposito, Rita Bentivoglio, Marina Frontali, Arianna Guidubaldi, Tamara Ialongo, Gioia Jacopini, Carla Piano, Silvia Romano, Francesco Soleti, Maria Spadaro, Monique S.E. van Hout, Marloes E. Verhoeven, Jeroen P.P. van Vugt, A. Marit de Weert, J.J.W. Bolwijn, M. Dekker, B. Kremer, K.L. Leenders, J.C.H. van Oostrom, Simon J.A. van den Bogaard, Eve M. Dumas, Ellen P. 't Hart, Berry Kremer, C.C.P. Verstappen, Olaf Aaserud, Jan Frich C, Ragnhild Wehus, Kathrine Bjørgo, Madeleine Fannemel, Per F. Gørvell, Eirin Lorentzen, Lars Retterstøl, Bodil Stokke, Inga Bjørnevoll, Sigrid Botne Sando, Artur Dziadkiewicz, Malgorzata Nowak, Piotr Robowski, Emilia Sitek, Jaroslaw Slawek, Witold Soltan, Michal Szinwelski, Magdalena Blaszcyk, Magdalena Boczarska-Jedynak, Ewelina Ciach-Wysocka, Agnieszka Gorzkowska, Barbara Jasinska-Myga, Gabriela Klodowska-Duda, Gregorz Opala, Daniel Stompel, Krzysztof Banaszkiewicz, Dorota Bocwinska, Kamila Bojakowska-Jaremek, Malgorzata Dec, Malgorzata Krawczyk, Monika Rudzinska, Elzbieta Szczygiel, Andrzej Szczudlik, Anna Wasielewska, Magdalena Wójcik, Anna Bryl, Anna Ciesielska, Aneta Klimberg, Jerzy Marcinkowski, Husam Samara, Justyna Sempolowicz, Anna Gogol, Piotr Janik, Hubert Kwiecinski, Zygmunt Jamrozik, Jakub Antczak, Katarzyna Jachinska, Wioletta Krysa, Maryla Rakowicz, Przemyslaw Richter, Rafal Rola, Danuta Ryglewicz, Halina Sienkiewicz-Jarosz, Iwona Stepniak, Anna Sulek, Elzbieta Zdzienicka, Karolina Zieora-Jakutowicz, Joaquim J Ferreira, Miguel Coelho, Tiago Mendes, Anabela Valadas, Carlos Andrade, Miguel Gago, Carolina Garrett, Maria Rosália Guerra, Carmen Durán Herrera, Patrocinio Moreno Garcia, Miquel Aguilar Barbera, Dolors Badenes Guia, Laura Casas Hernanz, Judit López Catena, Pilar Quiléz Ferrer, Ana Rojo Sebastián, Gemma Tome Carruesco, Jordi Bas, Núria Busquets, Matilde Calopa, Misericordia Floriach Robert, Celia Mareca Viladrich, Jesús Miguel Ruiz Idiago, Antonio Villa Riballo, Esther Cubo, Cecilia Gil Polo, Natividad Mariscal, Perez Jessica Rivadeneyra, Francisco Barrero, Blas Morales, María Fenollar, Rocío García-Ramos García, Paloma Ortega, Clara Villanueva, Javier Alegre, Mónica Bascuñana, Juan Garcia Caldentey, Marta Fatás Ventura, Guillermo García Ribas, Justo García de Yébenes, José Luis López-Sendón Moreno, Fernando Alonso Frech, Pedro J García Ruíz, Asunción Martínez-Descals, Rosa Guerrero, María José Saiz Artiga, Vicenta Sánchez, María Fuensanta Noguera Perea, Lorenza Fortuna, Salvadora Manzanares, Gema Reinante, María Martirio Antequera Torres, Laura Vivancos Moreau, Sonia González González, Luis Menéndez Guisasola, Carlos Salvador, Esther Suaréz San Martín, Inés Legarda Ramirez, Aranzazú Gorospe, Mónica Rodriguez Lopera, Penelope Navas Arques, María José Torres Rodríguez, Barbara Vives Pastor, Itziar Gaston, Maria Dolores Martinez-Jaurrieta, Jose Manuel Garcia Moreno, Carolina Mendez Lucena, Fatima Damas, Hermoso Eva Pacheco Cortegana, José Chacón Peña, Luis Redondo, Fátima Carrillo, María Teresa Cáceres, Pablo Mir, María José Lama Suarez, Laura Vargas-González, Maria E. Bosca, Francisco Castera Brugada, Juan Andres Burguera, Anabel Campos, Garcia Carmen Peiró Vilaplana, Peter Berglund, Radu Constantinescu, Gunnel Fredlund, Ulrika Høsterey-Ugander, Petra Linnsand, Liselotte Neleborn-Lingefjärd, Magnus Wentzel, Ghada Loutfi, Carina Olofsson, Eva-Lena Stattin, Laila Westman, Birgitta Wikström, Yanik Stebler, Alain Kaelin, Irene Romero, Michael Schüpbach, Sabine Weber Zaugg, Maria Hauer, Roman Gonzenbach, Hans H. Jung, Violeta Mihaylova, Jens Petersen, Roisin Jack, Kirsty Matheson, Zosia Miedzybrodzka, Daniela Rae, Sheila A Simpson, Fiona Summers, Alexandra Ure, Vivien Vaughan, Shahbana Akhtar, Jenny Crooks, Adrienne Curtis, Jenny de Souza, John Piedad, Hugh Rickards, Jan Wright, Elizabeth Coulthard, Louise Gethin, Beverley Hayward, Kasia Sieradzan, Matthew Armstrong, Roger A. Barker, Deidre O'Keefe, Anna Di Pietro, Kate Fisher, Anna Goodman, Susan Hill, Ann Kershaw, Sarah Mason, Nicole Paterson, Lucy Raymond, Rachel Swain, Natalie Valle Guzman, Monica Busse, Cynthia Butcher, Catherine Clenaghan, Sarah Hunt, Una Jones, Hanan Khalil, Michael Owen, Kathleen Price, Anne Rosser, Maureen Edwards, Carrie Ho, Teresa Hughes, Marie McGill, Pauline Pearson, Mary Porteous, Paul Smith, Peter Brockie, Jillian Foster, Nicola Johns, Sue McKenzie, Jean Rothery, Gareth Thomas, Shona Yates, Liz Burrows, Carol Chu, Amy Fletcher, Deena Gallantrae, Stephanie Hamer, Alison Harding, Stefan Klöppel, Alison Kraus, Fiona Laver, Monica Lewis, Mandy Longthorpe, Ivana Markova, Ashok Raman, Nicola Robertson, Mark Silva, Aileen Thomson, Sue Wild, Pam Yardumian, Carole Evans, Deena Gallentrae, Emma Hobson, Stuart Jamieson, Hannah Musgrave, Liz Rowett, Jean Toscano, Colin Bourne, Jackie Clapton, Carole Clayton, Heather Dipple, Dawn Freire-Patino, Janet Grant, Diana Gross, Caroline Hallam, Julia Middleton, Ann Murch, Catherine Thompson, Sundus Alusi, Rhys Davies, Kevin Foy, Emily Gerrans, Louise Pate, Thomasin Andrews, Andrew Dougherty, Charlotte Golding, Fred Kavalier, Hana Laing, Alison Lashwood, Dene Robertson, Deborah Ruddy, Alastair Santhouse, Anna Whaite, Stefania Bruno, Karen Doherty, Salman Haider, Davina Hensman, Nayana Lahiri, Marianne Novak, Aakta Patel, Elisabeth Rosser, Rachel Taylor, Thomas Warner, Edward Wild, Natalie Arran, Judith Bek, David Craufurd, Marianne Hare, Liz Howard, Susan Huson, Liz Johnson, Mary Jones, Helen Murphy, Emma Oughton, Lucy Partington-Jones, Dawn Rogers, Andrea Sollom, Julie Snowden, Cheryl Stopford, Jennifer Thompson, Iris Trender-Gerhard, Nichola Verstraelen, Leann Westmoreland, Richard Armstrong, Kathryn Dixon, Andrea H Nemeth, Gill Siuda, Ruth Valentine, David Harrison, Max Hughes, Andrew Parkinson, Beverley Soltysiak, Oliver Bandmann, Alyson Bradbury, Paul Gill, Helen Fairtlough, Kay Fillingham, Isabella Foustanos, Mbombe Kazoka, Kirsty O'Donovan, Cat Taylor, Katherine Tidswell, Oliver Quarrell, Puay Ngoh Lau, Emmanul Pica, Louis Tan, Univ Angers, Okina, Moss, Davina J. Hensman, Tabrizi, Sarah J, Mead, Simon, Kitty, Lo, Pardiã±as, Antonio F, Holmans, Peter, Jones, Lesley, Langbehn, Dougla, Coleman, A., Santos, R. Dar, Decolongon, J., Sturrock, A., Bardinet, E., Ret, C. Jauff, Justo, D., Lehericy, S., Marelli, C., Nigaud, K., Valabrãgue, R., van den Bogaard, S. J. A., Dumas, E. M., van der Grond, J., T'Hart, E. P., Jurgens, C., Witjes-Ane, M. -. N., Arran, N., Callaghan, J., Stopford, C., Frost, C., Jones, R., Hobbs, N., Lahiri, N., Ordidge, R., Owen, G., Pepple, T., Read, J., Say, M., Wild, E., Patel, A., Fox, N. C., Gibbard, C., Malone, I., Crawford, H., Whitehead, D., Keenan, S., Cash, D. M., Berna, C., Bechtel, N., Bohlen, S., Man, A. Hoff, Kraus, P., Axelson, E., Wang, C., Acharya, T., Lee, S., Monaco, W., Campbell, C., Queller, S., Whitlock, K., Campbell, M., Frajman, E., Milchman, C., O'Regan, A., Labuschagne, I., Stout, J., Landwehrmeyer, B., Craufurd, D., Scahill, R., Hicks, S., Kennard, C., Johnson, H., Tobin, A., Rosas, H. D., Reilmann, R., Borowsky, B., Pourchot, C., Andrews, S. C., Bachoud-Lévi, Anne-Catherine, Bentivoglio, Anna Rita, Biunno, Ida, Bonelli, Raphael, Burgunder, Jean-Marc, Dunnett, Stephen, Ferreira, Joaquim, Handley, Olivia, Heiberg, Arvid, Illmann, Torsten, Landwehrmeyer, G. Bernhard, Levey, Jamie, Ramos-Arroyo, Maria A., Nielsen, Jã¸rgen, Koivisto, Susana Pro, Pã¤ivã¤rinta, Markku, Roos, Raymund A. C., Sebastiã¡n, A. Rojo, Tabrizi, Sarah, Vandenberghe, Wim, Verellen-Dumoulin, Christine, Uhrova, Tereza, Wahlstrã¶m, Jan, Zaremba, Jacek, Baake, Verena, Barth, Katrin, Garde, Monica Bascuñana, Betz, Sabrina, Bos, Reineke, Callaghan, Jenny, Come, Adrien, Guedes, Leonor Correia, Ecker, Daniel, Finisterra, Ana Maria, Fullam, Ruth, Gilling, Mette, Gustafsson, Lena, Handley, Olivia J, Hvalstedt, Carina, Held, Christine, Koppers, Kerstin, Lamanna, Claudia, Laurã , Matilde, Descals, Asunción Martínez, Martinez-Horta, Saã¼l, Mestre, Tiago, Minster, Sara, Monza, Daniela, Mã¼tze, Lisanne, Oehmen, Martin, Orth, Michael, Padieu, Hã©lãne, Paterski, Laurent, Peppa, Nadia, Di Renzo, Martina, Rialland, Amandine, Rã¸ren, Niini, Å aå¡inkovã¡, Pavla, Timewell, Erika, Townhill, Jenny, Cubillo, Patricia Trigo, da Silva, Wildson Vieira, van Walsem, Marleen R, Whalstedt, Carina, Witjes-Ané, Marie-Noelle, Witkowski, Grzegorz, Wright, Abigail, Zielonka, Daniel, Zielonka, Eugeniusz, Zinzi, Paola, Bonelli, Raphael M., Lilek, Sabine, Hecht, Karen, Herranhof, Brigitte, Holl, Anna, Kapfhammer, Hans-Peter, Koppitz, Michael, Magnet, Marku, Mã¼ller, Nicole, Otti, Daniela, Painold, Annamaria, Reisinger, Karin, Scheibl, Monika, Schã¶ggl, Helmut, Ullah, Jasmin, Braunwarth, Eva-Maria, Brugger, Florian, Buratti, Lisa, Hametner, Eva-Maria, Hepperger, Caroline, Holas, Christiane, Hotter, Anna, Hussl, Anna, Mã¼ller, Christoph, Poewe, Werner, Seppi, Klau, Sprenger, Fabienne, Wenning, Gregor, Boogaerts, Andrea, Calmeyn, Godelinde, Delvaux, Isabelle, Liessens, Dirk, Somers, Nele, Dupuit, Michel, Minet, Cã©cile, van Paemel, Dominique, Ribaã¯, Pascale, van Reijen, Dimphna, Klempã­r, Jirã­, Majerovã¡, Veronika, Roth, Jan, Stã¡rkovã¡, Irena, Hjermind, Lena E., Jacobsen, Oda, Nielsen, Jørgen E., Larsen, Ida Unmack, Vinther-Jensen, Tua, Hiivola, Heli, Hyppã¶nen, Hannele, Martikainen, Kirsti, Tuuha, Katri, Allain, Philippe, Bonneau, Dominique, Bost, Marie, Gohier, Bã©nã©dicte, Guã©rid, Marie-Anne, Olivier, Audrey, Prundean, Adriana, Scherer-Gagou, Clarisse, Verny, Christophe, Babiloni, Blandine, Debruxelles, Sabrina, Duchã©, Charlotte, Goizet, Cyril, Jameau, Laetitia, Lafoucriãre, Danielle, Spampinato, Umberto, Barthã©lã©my, Rekha, De Bruycker, Christelle, Carette, Maryline Cabaret Anne-Sophie, Defebvre, Eric Decorte Luc, Delliaux, Marie, Delval, Arnaud, Destee, Alain, Dujardin, Kathy, Lemaire, Marie-HélÃne, Manouvrier, Sylvie, Peter, Mireille, Plomhouse, Lucie, Sablonniãre, Bernard, Simonin, Clã©mence, Thibault-Tanchou, Stã©phanie, Vuillaume, Isabelle, Bellonet, Marcellin, Berrissoul, Hassan, Blin, Stã©phanie, Courtin, Franã§oise, Duru, Cã©cile, Fasquel, Vã©ronique, Godefroy, Olivier, Krystkowiak, Pierre, Mantaux, Bã©atrice, Roussel, Martine, Wannepain, Sandrine, Azulay, Jean-Philippe, Delfini, Marie, Eusebio, Alexandre, Fluchere, Frã©dã©rique, Mundler, Laura, Anheim, Mathieu, Juliã©, Celine, Boukbiza, Ouhaid Lagha, Longato, Nadine, Rudolf, Gabrielle, Tranchant, Christine, Zimmermann, Marie-Agathe, Kosinski, Christoph Michael, Milkereit, Eva, Probst, Daniela, Reetz, Kathrin, Sass, Christian, Schiefer, Johanne, Schlangen, Christiane, Werner, Cornelius J., Gelderblom, Harald, Priller, Josef, Prã¼ã , Harald, Spruth, Eike Jakob, Ellrichmann, Gisa, Herrmann, Lennard, Hoffmann, Rainer, Kaminski, Barbara, Kotz, Peter, Prehn, Christian, Saft, Carsten, Lange, Herwig, Maiwald, Robert, Lã¶hle, Matthia, Maass, Antonia, Schmidt, Simone, Bosredon, Cecile, Storch, Alexander, Wolz, Annett, Wolz, Martin, Capetian, Philipp, Lambeck, Johann, Zucker, Birgit, Boelmans, Kai, Ganos, Christo, Heinicke, Walburgi, Hidding, Ute, Lewerenz, Jan, Mã¼nchau, Alexander, Schmalfeld, Jenny, Stubbe, Lar, Zittel, Simone, Diercks, Gabriele, Dressler, Dirk, Gorzolla, Heike, Schrader, Christoph, Tacik, Pawel, Ribbat, Michael, Longinus, Bernhard, Bã¼rk, Katrin, Mã¶ller, Jens Carsten, Rissling, Ida, Mã¼hlau, Mark, Peinemann, Alexander, Stã¤dtler, Michael, Weindl, Adolf, Winkelmann, Juliane, Ziegler, Cornelia, Bechtel, Natalie, Beckmann, Heike, Bohlen, Stefan, Hã¶lzner, Eva, Reilmann, Ralf, Rohm, Stefanie, Rumpf, Silke, Schepers, Sigrun, Weber, Natalia, Dose, Matthia, Leythã¤user, Gabriele, Marquard, Ralf, Raab, Tina, Wiedemann, Alexandra, Buck, Andrea, Connemann, Julia, Geitner, Carolin, Kesse, Andrea, Landwehrmeyer, Bernhard, Lang, Christina, Lezius, Franziska, Nepper, Solveig, Niess, Anke, Schneider, Ariane, Schwenk, Daniela, Sã¼ã muth, Sigurd, Trautmann, Sonja, Weydt, Patrick, Cormio, Claudia, Sciruicchio, Vittorio, Serpino, Claudia, de Tommaso, Marina, Capellari, Sabina, Cortelli, Pietro, Galassi, Roberto, Rizzo, Giovanni, Poda, Roberto, Scaglione, Cesa, Bertini, Elisabetta, Ghelli, Elena, Ginestroni, Andrea, Massaro, Francesca, Mechi, Claudia, Paganini, Marco, Piacentini, Silvia, Pradella, Silvia, Romoli, Anna Maria, Sorbi, Sandro, Abbruzzese, Giovanni, di Poggio, Monica Bandettini, Ferrandes, Giovanna, Mandich, Paola, Roberta, Marchese, Albanese, Alberto, Di Bella, Daniela, Castaldo, Anna, Di Donato, Stefano, Gellera, Cinzia, Genitrini, Silvia, Mariotti, Caterina, Nanetti, Lorenzo, Paridi, Dominga, Soliveri, Paola, Tomasello, Chiara, De Michele, Giuseppe, Di Maio, Luigi, Massarelli, Marco, Peluso, Silvio, Roca, Alessandro, Russo, Cinzia Valeria, Salvatore, Elena, Sorrentino, Pierpaolo, Amico, Enrico, Favellato, Mariagrazia, Griguoli, Annamaria, Mazzante, Irene, Petrollini, Martina, Squitieri, Ferdinando, D'Alessio, Barbara, Esposito, Chiara, Bentivoglio, Rita, Frontali, Marina, Guidubaldi, Arianna, Ialongo, Tamara, Jacopini, Gioia, Piano, Carla, Romano, Silvia, Soleti, Francesco, Spadaro, Maria, van Hout, Monique S. E., Verhoeven, Marloes E., van Vugt, Jeroen P. P., de Weert, A. Marit, Bolwijn, J. J. W., Dekker, M., Kremer, B., Leenders, K. L., van Oostrom, J. C. H., van den Bogaard, Simon J. A., Dumas, Eve M., â t Hart, Ellen P., Kremer, Berry, Verstappen, C. C. P., Aaserud, Olaf, Jan Frich, C., Wehus, Ragnhild, Bjã¸rgo, Kathrine, Fannemel, Madeleine, Gã¸rvell, Per F., Lorentzen, Eirin, Retterstã¸l, Lar, Stokke, Bodil, Bjã¸rnevoll, Inga, Sando, Sigrid Botne, Dziadkiewicz, Artur, Nowak, Malgorzata, Robowski, Piotr, Sitek, Emilia, Slawek, Jaroslaw, Soltan, Witold, Szinwelski, Michal, Blaszcyk, Magdalena, Boczarska-Jedynak, Magdalena, Ciach-Wysocka, Ewelina, Gorzkowska, Agnieszka, Jasinska-Myga, Barbara, Klodowska-Duda, Gabriela, Opala, Gregorz, Stompel, Daniel, Banaszkiewicz, Krzysztof, Bocwinska, Dorota, Bojakowska-Jaremek, Kamila, Dec, Malgorzata, Krawczyk, Malgorzata, Rudzinska, Monika, Szczygiel, Elzbieta, Szczudlik, Andrzej, Wasielewska, Anna, Wã³jcik, Magdalena, Bryl, Anna, Ciesielska, Anna, Klimberg, Aneta, Marcinkowski, Jerzy, Samara, Husam, Sempolowicz, Justyna, Gogol, Anna, Janik, Piotr, Kwiecinski, Hubert, Jamrozik, Zygmunt, Antczak, Jakub, Jachinska, Katarzyna, Krysa, Wioletta, Rakowicz, Maryla, Richter, Przemyslaw, Rola, Rafal, Ryglewicz, Danuta, Sienkiewicz-Jarosz, Halina, Stepniak, Iwona, Sulek, Anna, Zdzienicka, Elzbieta, Zieora-Jakutowicz, Karolina, Ferreira, Joaquim J, Coelho, Miguel, Mendes, Tiago, Valadas, Anabela, Andrade, Carlo, Gago, Miguel, Garrett, Carolina, Guerra, Maria Rosália, Herrera, Carmen Durán, Garcia, Patrocinio Moreno, Barbera, Miquel Aguilar, Guia, Dolors Badene, Hernanz, Laura Casa, Catena, Judit López, Ferrer, Pilar Quiléz, Sebastiã¡n, Ana Rojo, Carruesco, Gemma Tome, Bas, Jordi, Busquets, Nãºria, Calopa, Matilde, Robert, Misericordia Floriach, Viladrich, Celia Mareca, Idiago, Jesús Miguel Ruiz, Riballo, Antonio Villa, Cubo, Esther, Polo, Cecilia Gil, Mariscal, Natividad, Rivadeneyra, Perez Jessica, Barrero, Francisco, Morales, Bla, Fenollar, Marã­a, Garcã­a, Rocío García-Ramo, Ortega, Paloma, Villanueva, Clara, Alegre, Javier, Bascuã±ana, Mã³nica, Caldentey, Juan Garcia, Ventura, Marta Fatá, Ribas, Guillermo García, de Yébenes, Justo García, Moreno, José Luis López-Sendón, Frech, Fernando Alonso, Ruã­z, Pedro J. García, Martínez-Descals, Asunciã³n, Guerrero, Rosa, Artiga, María José Saiz, Sã¡nchez, Vicenta, Perea, María Fuensanta Noguera, Fortuna, Lorenza, Manzanares, Salvadora, Reinante, Gema, Torres, María Martirio Antequera, Moreau, Laura Vivanco, González González, Sonia, Guisasola, Luis Menéndez, Salvador, Carlo, Martã­n, Esther Suaréz San, Ramirez, Inés Legarda, Gorospe, Aranzazãº, Lopera, Mónica Rodriguez, Arques, Penelope Nava, Rodrã­guez, María José Torre, Pastor, Barbara Vive, Gaston, Itziar, Martinez-Jaurrieta, Maria Dolore, Moreno, Jose Manuel Garcia, Lucena, Carolina Mendez, Damas, Fatima, Cortegana, Hermoso Eva Pacheco, Peã±a, José Chacón, Redondo, Lui, Carrillo, Fã¡tima, Teresa Cáceres, Marã­a, Mir, Pablo, Suarez, María José Lama, Vargas-González, Laura, Bosca, Maria E., Brugada, Francisco Castera, Burguera, Juan Andre, Campos, Anabel, Vilaplana, Garcia Carmen Peiró, Berglund, Peter, Constantinescu, Radu, Fredlund, Gunnel, Høsterey-Ugander, Ulrika, Linnsand, Petra, Neleborn-Lingefjärd, Liselotte, Wentzel, Magnu, Loutfi, Ghada, Olofsson, Carina, Stattin, Eva-Lena, Westman, Laila, Wikstrã¶m, Birgitta, Stebler, Yanik, Kaelin, Alain, Romero, Irene, Schã¼pbach, Michael, Weber Zaugg, Sabine, Hauer, Maria, Gonzenbach, Roman, Jung, Hans H., Mihaylova, Violeta, Petersen, Jen, Jack, Roisin, Matheson, Kirsty, Miedzybrodzka, Zosia, Rae, Daniela, Simpson, Sheila A, Summers, Fiona, Ure, Alexandra, Vaughan, Vivien, Akhtar, Shahbana, Crooks, Jenny, Curtis, Adrienne, de Souza, Jenny, Piedad, John, Rickards, Hugh, Wright, Jan, Coulthard, Elizabeth, Gethin, Louise, Hayward, Beverley, Sieradzan, Kasia, Armstrong, Matthew, Barker, Roger A., O'Keefe, Deidre, Di Pietro, Anna, Fisher, Kate, Goodman, Anna, Hill, Susan, Kershaw, Ann, Mason, Sarah, Paterson, Nicole, Raymond, Lucy, Swain, Rachel, Guzman, Natalie Valle, Busse, Monica, Butcher, Cynthia, Clenaghan, Catherine, Hunt, Sarah, Jones, Una, Khalil, Hanan, Owen, Michael, Price, Kathleen, Rosser, Anne, Edwards, Maureen, Carrie, Ho, Hughes, Teresa, Mcgill, Marie, Pearson, Pauline, Porteous, Mary, Smith, Paul, Brockie, Peter, Foster, Jillian, Johns, Nicola, Mckenzie, Sue, Rothery, Jean, Thomas, Gareth, Yates, Shona, Burrows, Liz, Chu, Carol, Fletcher, Amy, Gallantrae, Deena, Hamer, Stephanie, Harding, Alison, Klã¶ppel, Stefan, Kraus, Alison, Laver, Fiona, Lewis, Monica, Longthorpe, Mandy, Markova, Ivana, Raman, Ashok, Robertson, Nicola, Silva, Mark, Thomson, Aileen, Wild, Sue, Yardumian, Pam, Evans, Carole, Gallentrae, Deena, Hobson, Emma, Jamieson, Stuart, Musgrave, Hannah, Rowett, Liz, Toscano, Jean, Bourne, Colin, Clapton, Jackie, Clayton, Carole, Dipple, Heather, Freire-Patino, Dawn, Grant, Janet, Gross, Diana, Hallam, Caroline, Middleton, Julia, Murch, Ann, Thompson, Catherine, Alusi, Sundu, Davies, Rhy, Foy, Kevin, Gerrans, Emily, Pate, Louise, Andrews, Thomasin, Dougherty, Andrew, Golding, Charlotte, Kavalier, Fred, Laing, Hana, Lashwood, Alison, Robertson, Dene, Ruddy, Deborah, Santhouse, Alastair, Whaite, Anna, Bruno, Stefania, Doherty, Karen, Haider, Salman, Hensman, Davina, Lahiri, Nayana, Novak, Marianne, Patel, Aakta, Rosser, Elisabeth, Taylor, Rachel, Warner, Thoma, Wild, Edward, Arran, Natalie, Bek, Judith, Craufurd, David, Hare, Marianne, Howard, Liz, Huson, Susan, Johnson, Liz, Jones, Mary, Murphy, Helen, Oughton, Emma, Partington-Jones, Lucy, Rogers, Dawn, Sollom, Andrea, Snowden, Julie, Stopford, Cheryl, Thompson, Jennifer, Trender-Gerhard, Iri, Verstraelen, Nichola, Westmoreland, Leann, Armstrong, Richard, Dixon, Kathryn, Nemeth, Andrea H, Siuda, Gill, Valentine, Ruth, David, Harrison, Hughes, Max, Parkinson, Andrew, Soltysiak, Beverley, Bandmann, Oliver, Bradbury, Alyson, Gill, Paul, Fairtlough, Helen, Fillingham, Kay, Foustanos, Isabella, Kazoka, Mbombe, O'Donovan, Kirsty, Taylor, Cat, Tidswell, Katherine, Quarrell, Oliver, Lau, Puay Ngoh, Pica, Emmanul, Tan, Louis, Amsterdam Neuroscience - Neurodegeneration, Neurology, Biologie Neurovasculaire et Mitochondriale Intégrée (BNMI), Université d'Angers (UA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Moss, Davina J Hensman, Lo, Kitty, Pardiñas, Antonio F, Santos, R Dar, Ret, C Jauff, Valabrègue, R., Witjes-Ane, M. -N., Man, A Hoff, Bachoud-Lévi, Anne-Catherine, Nielsen, Jørgen, Päivärinta, Markku, Sebastián, A Rojo, Wahlström, Jan, Garde, Monica Bascuñana, Laurà, Matilde, Descals, Asunción Martínez, Martinez-Horta, Saül, Mütze, Lisanne, Padieu, Hélène, Røren, Niini, Šašinková, Pavla, Witjes-Ané, Marie-Noelle, Müller, Nicole, Schöggl, Helmut, Müller, Christoph, Minet, Cécile, Ribaï, Pascale, Klempír, Jirí, Majerová, Veronika, Stárková, Irena, Nielsen, Jørgen E., Hyppönen, Hannele, Gohier, Bénédicte, Guérid, Marie-Anne, Duché, Charlotte, Lafoucrière, Danielle, Barthélémy, Rekha, Lemaire, Marie-Hélène, Sablonnière, Bernard, Simonin, Clémence, Thibault-Tanchou, Stéphanie, Blin, Stéphanie, Courtin, Françoise, Duru, Cécile, Fasquel, Véronique, Mantaux, Béatrice, Fluchere, Frédérique, Julié, Celine, Prüß, Harald, Löhle, Matthia, Münchau, Alexander, Bürk, Katrin, Möller, Jens Carsten, Mühlau, Mark, Städtler, Michael, Hölzner, Eva, Leythäuser, Gabriele, Süßmuth, Sigurd, Marchese, Roberta, DI MAIO, Luigi, ’t Hart, Ellen P., Bjørgo, Kathrine, Gørvell, Per F., Retterstøl, Lar, Bjørnevoll, Inga, Wójcik, Magdalena, Guerra, Maria Rosália, Herrera, Carmen Durán, Catena, Judit López, Ferrer, Pilar Quiléz, Sebastián, Ana Rojo, Busquets, Núria, Idiago, Jesús Miguel Ruiz, Fenollar, María, García, Rocío García-Ramo, Bascuñana, Mónica, Ventura, Marta Fatá, Ribas, Guillermo García, de Yébenes, Justo García, Moreno, José Luis López-Sendón, Ruíz, Pedro J García, Martínez-Descals, Asunción, Artiga, María José Saiz, Sánchez, Vicenta, Perea, María Fuensanta Noguera, Torres, María Martirio Antequera, González González, Sonia, Guisasola, Luis Menéndez, Martín, Esther Suaréz San, Ramirez, Inés Legarda, Gorospe, Aranzazú, Lopera, Mónica Rodriguez, Rodríguez, María José Torre, Peña, José Chacón, Carrillo, Fátima, Teresa Cáceres, María, Suarez, María José Lama, Vargas-González, Laura, Vilaplana, Garcia Carmen Peiró, Høsterey-Ugander, Ulrika, Neleborn-Lingefjärd, Liselotte, Wikström, Birgitta, Schüpbach, Michael, Ho, Carrie, Klöppel, Stefan, Harrison, David, Cardiff University, University of Iowa [Iowa City], University of British Columbia (UBC), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), UCL, Institute of Neurology [London], National Oceanography Centre [Southampton] (NOC), University of Southampton, Center for NeuroImaging Research-Human MRI Neuroimaging core facility for clinical research [ICM Paris] (CENIR), Institut du Cerveau = Paris Brain Institute (ICM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-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)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-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)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), IFR de Neuroimagerie Fonctionnelle (IFR 49), Commissariat à l'énergie atomique et aux énergies alternatives (CEA), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), CHU Amiens-Picardie, Laboratoire de Neurosciences Fonctionnelles et Pathologies - UR UPJV 4559 (LNFP), Université de Picardie Jules Verne (UPJV), CHirurgie, IMagerie et REgénération tissulaire de l’extrémité céphalique - Caractérisation morphologique et fonctionnelle - UR UPJV 7516 (CHIMERE), TRACK-HD investigators, Pardinas, Antonio F, Langbehn, Douglas, Lee, S Hong, TRACK-HD Investigators, and REGISTRY Investigators

Subjects
0301 basic medicine, Oncology, Registrie, Genome-wide association study, Longitudinal Studie, Disease, Bioinformatics, Severity of Illness Index, Principal Component Analysi, Longitudinal Studies, Registries, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, Huntington disease, DNA-Binding Proteins, Settore MED/26 - NEUROLOGIA, Adult, Genome-Wide Association Study, Humans, Huntington Disease, MutS Homolog 3 Protein, Principal Component Analysis, Disease Progression, Neurology (clinical), huntingtin gene, age of onest, Huntington’s disease, Human, medicine.medical_specialty, cag repeat, instability, DNA-Binding Protein, Clinical Neurology, Principal component analysis, Single-nucleotide polymorphism, Biology, 03 medical and health sciences, Huntington's disease, Internal medicine, medicine, SNP, genome-wide association study, medicine.disease, R1, meta-analysis, Minor allele frequency, 030104 developmental biology, Age of onset, Trinucleotide repeat expansion, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

Background Huntington's disease is caused by a CAG repeat expansion in the huntingtin gene, HTT. Age at onset has been used as a quantitative phenotype in genetic analysis looking for Huntington's disease modifiers, but is hard to define and not always available. Therefore, we aimed to generate a novel measure of disease progression and to identify genetic markers associated with this progression measure. Methods We generated a progression score on the basis of principal component analysis of prospectively acquired longitudinal changes in motor, cognitive, and imaging measures in the 218 indivduals in the TRACK-HD cohort of Huntington's disease gene mutation carriers (data collected 2008–11). We generated a parallel progression score using data from 1773 previously genotyped participants from the European Huntington's Disease Network REGISTRY study of Huntington's disease mutation carriers (data collected 2003–13). We did a genome-wide association analyses in terms of progression for 216 TRACK-HD participants and 1773 REGISTRY participants, then a meta-analysis of these results was undertaken. Findings Longitudinal motor, cognitive, and imaging scores were correlated with each other in TRACK-HD participants, justifying use of a single, cross-domain measure of disease progression in both studies. The TRACK-HD and REGISTRY progression measures were correlated with each other (r=0·674), and with age at onset (TRACK-HD, r=0·315; REGISTRY, r=0·234). The meta-analysis of progression in TRACK-HD and REGISTRY gave a genome-wide significant signal (p=1·12 × 10−10) on chromosome 5 spanning three genes: MSH3, DHFR, and MTRNR2L2. The genes in this locus were associated with progression in TRACK-HD (MSH3 p=2·94 × 10−8 DHFR p=8·37 × 10−7 MTRNR2L2 p=2·15 × 10−9) and to a lesser extent in REGISTRY (MSH3 p=9·36 × 10−4 DHFR p=8·45 × 10−4 MTRNR2L2 p=1·20 × 10−3). The lead single nucleotide polymorphism (SNP) in TRACK-HD (rs557874766) was genome-wide significant in the meta-analysis (p=1·58 × 10−8), and encodes an aminoacid change (Pro67Ala) in MSH3. In TRACK-HD, each copy of the minor allele at this SNP was associated with a 0·4 units per year (95% CI 0·16–0·66) reduction in the rate of change of the Unified Huntington's Disease Rating Scale (UHDRS) Total Motor Score, and a reduction of 0·12 units per year (95% CI 0·06–0·18) in the rate of change of UHDRS Total Functional Capacity score. These associations remained significant after adjusting for age of onset. Interpretation The multidomain progression measure in TRACK-HD was associated with a functional variant that was genome-wide significant in our meta-analysis. The association in only 216 participants implies that the progression measure is a sensitive reflection of disease burden, that the effect size at this locus is large, or both. Knockout of Msh3 reduces somatic expansion in Huntington's disease mouse models, suggesting this mechanism as an area for future therapeutic investigation. Funding The European Commission FP7 NeurOmics project; CHDI Foundation; the Medical Research Council UK; the Brain Research Trust; and the Guarantors of Brain.

Published
2017
Full Text
View/download PDF

4. Apathy in untreated early-stage Parkinson disease: Relationship with other non-motor symptoms

Author

Anne-Sophie Carette, Alain Duhamel, Carole Langlois, Lucie Plomhause, Kathy Dujardin, Luc Defebvre, and Marie Delliaux

Subjects
medicine.medical_specialty, Anhedonia, Disease, Disease Relationship, Neurology, Internal medicine, Concomitant, medicine, Non motor, Apathy, Neurology (clinical), medicine.symptom, Stage (cooking), Psychiatry, Psychology, Depression (differential diagnoses)
Abstract

Apathy is a frequent and disabling behavioral disorder in patients with Parkinson's disease (PD). Its prevalence in treatment-naive patients with early-stage PD has not been extensively investigated. Moreover, whether apathy is related to other non-motor symptoms in early-stage PD is unknown. Our objective was to determine the prevalence and features of apathy and associated factors in a group of treatment-naive patients with early-stage PD. Ninety-five treatment-naive patients with early-stage PD participated in the study. Apathy, depression, motor symptoms, and overall cognitive efficiency were assessed. The presence of the main non-motor symptoms was checked during a detailed clinical interview. Group comparisons were carried out to investigate the association with apathy. Eighteen patients (18.95%) were diagnosed as apathetic, and five of the latter had concomitant depression. Apathetic patients had significantly more severe motor symptoms (P

Published
2014
Full Text
View/download PDF

5. Assessing apathy in everyday clinical practice with the short-form Lille Apathy Rating Scale

Author

Kathy Dujardin, Marie Delliaux, Pascal Sockeel, Luc Defebvre, and Anne-Sophie Carette

Subjects
medicine.medical_specialty, Poison control, Human factors and ergonomics, Suicide prevention, Occupational safety and health, Neurology, Rating scale, Injury prevention, medicine, Apathy, Neurology (clinical), medicine.symptom, Psychology, Psychiatry, Reliability (statistics)
Abstract

Background: Apathy is an invalidating behavioral disorder that must always be screened for and assessed in patients with Parkinson’s disease (PD). The Lille Apathy Rating Scale (LARS) has been validated in several different contexts, but the lengthy administration time means that it is mostly recommended for research use. The aim of the present study was to validate a short form of the LARS for use in everyday practice. Methods: In total, 416 patients with PD and 56 healthy controls participated in the study. Apathy, depression, motor symptoms, and overall cognitive efficiency were assessed. The most discriminant items of the LARS for apathy detection were selected using multiple indicators. Results: A subset of 12 items (constituting the shortform LARS) showed the best convergence. Concurrent and criterion-related validity, internal consistency, testretest reliability, and inter-rater reliability were very good. Conclusions: The short-form LARS is a reliable, practical, patient interview-based instrument for assessing apathy in everyday clinical practice. V C 2013 Interna

Published
2013
Full Text
View/download PDF

6. Apathy may herald cognitive decline and dementia in Parkinson's disease

Author

Marie Delliaux, Pascal Sockeel, Alain Destée, Luc Defebvre, and Kathy Dujardin

Subjects
medicine.medical_specialty, Parkinson's disease, Cognitive disorder, Cognition, medicine.disease, Neurology, medicine, Dementia, Apathy, Neurology (clinical), Effects of sleep deprivation on cognitive performance, Cognitive decline, medicine.symptom, Psychiatry, Psychology, Executive dysfunction
Abstract

Apathy is usually defined as a lack of motivation. It may occur as part of another disorder (notably depression and dementia) or as an isolated syndrome. In Parkinson's disease (PD), apathy is common and several studies have reported an association between this condition and more severe cognitive symptoms, such as executive dysfunction. However, this association has not been thoroughly investigated. The aim of this study (in nondepressed, nondemented PD patients) was to examine whether or not cognitive decline and/or dementia occurred more frequently in apathetic subjects than in nonapathetic subjects. Forty consecutive PD patients participated in the study (20 with apathy and 20 without). None of the subjects were either demented or depressed at the time of study entry. The patients' cognitive functions were extensively assessed twice: at study entry and after an 18-month follow-up period. At study entry, the apathetic PD patients had significantly lower global cognitive status and executive function scores than the nonapathetic subjects. After a median period of 18 months, the rate of conversion to dementia was found to be significantly higher in the apathetic group than in the nonapathetic group (8 of 20 and 1 of 20, respectively). Even in nondemented patients, the decrease over time in cognitive performance (mainly executive function but also memory impairment) was significantly greater in apathetic subjects than in nonapathetic subjects. These findings suggest that in nondemented, nondepressed PD patients, apathy may be a predictive factor for dementia and cognitive decline over time.

Published
2009
Full Text
View/download PDF

7. REM sleep behaviour disorder and visuoperceptive dysfunction: a disorder of the ventral visual stream?

Author

Luc Defebvre, Kathy Dujardin, Philippe Derambure, Muriel Boucart, Delphine Pins, Marie Delliaux, Ana Marques, and Christelle Charley Monaca

Subjects
Male, medicine.medical_specialty, Parkinson's disease, Neurology, Polysomnography, Vision Disorders, Rapid eye movement sleep, REM Sleep Behavior Disorder, Neurological disorder, Neuropsychological Tests, Audiology, Contrast Sensitivity, Perceptual Disorders, Central nervous system disease, Disability Evaluation, Degenerative disease, medicine, Humans, Attention, Visual Pathways, Aged, Sleep disorder, medicine.diagnostic_test, Brain, Parkinson Disease, Middle Aged, medicine.disease, Temporal Lobe, Pattern Recognition, Visual, Female, Neurology (clinical), Cognition Disorders, Psychology, Neuroscience, Photic Stimulation
Abstract

In idiopathic rapid eye movement sleep behaviour disorder (RBD), an association with visuoperceptive disorders has been described. However, such an association has not been clearly established in RBD secondary to Parkinson's disease (PD). We compared visuoperceptive function in four groups of non-demented patients (parkinsonian patients with or without RBD, patients with idiopathic RBD and control participants) via a procedure enabling the analysis of the various components of visual information processing and in order to answer the following question: is RBD associated with visuoperceptive and/or attentional disorders in PD and, if so, where is the dysfunction located along the visual pathway? Sensorial aspects of visual information were evaluated using a contrast sensitivity test, perceptual aspects were assessed using a contour-based object identification test and visual attention was measured in an attentional capture paradigm. The diagnosis of RBD was confirmed by polysomnography. We observed a higher object identification threshold (OIT) (1) in PD patients with RBD compared with PD patients without RBD and with controls and (2) in idiopathic RBD patients compared with controls. There were no significant OIT differences between PD patients with RBD and idiopathic RBD patients or between PD patients without RBD and controls. We did not find any significant inter-group differences in any of the other visuoperceptive tests. RBD, idiopathic or secondary to PD, is associated with perceptual closure dysfunction. Our results suggest that this perceptual dysfunction is specifically associated with RBD and may be related to a non-dopaminergic impairment.

Published
2009
Full Text
View/download PDF

8. Effect of external cueing on gait in Huntington's disease

Author

Alain Destée, Arnaud Delval, Pierre Krystkowiak, Luc Defebvre, Philippe Derambure, Marie Delliaux, and J.L. Blatt

Subjects
Dual-task paradigm, medicine.medical_specialty, Elementary cognitive task, Cognition, Metronome, law.invention, Task (project management), Physical medicine and rehabilitation, Gait (human), Neurology, law, medicine, Physical therapy, Neurology (clinical), Cadence, Set (psychology), Psychology
Abstract

In Huntington's disease (HD) patients, gait is characterized by a timing disorder with marked intraindividual variability in temporal gait parameters (caused by the presence of both hyperkinetic and hypokinetic features). We sought to determine the influence of use of a metronome on gait parameters in patients simultaneously performing motor or cognitive tasks that required attentional resources. The objective is to evaluate the influence of rhythmic cues on gait interference during self-regulated walking and a dual task paradigm in HD. Fifteen HD patients and 15 paired controls were asked to walk and simultaneously perform another motor task (carrying a tray with four full glasses) or a cognitive task (counting backwards). We evaluated the effect of a metronome (set at 100% and 120% of the subject's self-determined cadence) in three different task conditions (gait alone, gait + motor task, gait + cognitive task). The use of auditory cues during free gait and dual tasks did not improve kinematic parameters in HD patients, in contrast to the situation for control subjects (improvement in gait speed and cadence but not stride length when the metronome was set at 120% in all conditions). HD patients have difficulty in synchronizing their footsteps with a metronome, mainly due to attentional deficits.

Published
2008
Full Text
View/download PDF

9. The Lille Apathy Rating Scale: Validation of a caregiver-based version

Author

Luc Defebvre, Kathy Dujardin, Alain Destée, Pascal Sockeel, and Marie Delliaux

Subjects
medicine.medical_specialty, Neurology, Cronbach's alpha, Rating scale, Internal consistency, Structured interview, medicine, Apathy, Neurology (clinical), medicine.symptom, Psychiatry, Psychology, Clinical psychology
Abstract

Apathy is reported in 16.5% to 70% of Parkinson's disease (PD) patients. Our recently developed Lille Apathy Rating Scale (LARS) has been specifically validated for patient-based assessment of apathy in PD. The aim of the present study was to validate a caregiver-based version of the LARS. Sixty consecutive PD patients and their respective caregivers participated in the study. An informant-based version of the LARS (LARS-i) was developed to rate apathy via a caregiver-based structured interview. Apathy was also assessed in a patient-based interview using the LARS and the informant- and clinician-rated versions of the Apathy Evaluation Scale (AES). Cronbach's alpha and standardized alpha coefficients were 0.872 and 0.877, respectively, and the split-half reliability was 0.901 (revealing good internal consistency). The test-retest and inter-rater reliability values were 0.960 and 0.996, respectively. Criterion-related validity (according to an independent, expert diagnosis) was good. Scores on the LARS and the LARS-i were highly correlated. However, apathy was rated significantly more severely by the caregiver than by the patient. This difference was significantly higher for demented than nondemented PD patients. The LARS-i was seen to have excellent psychometric properties and appears to be valid for use in PD with respect to the patient-based LARS and the informant- and clinician-rated versions of the AES. © 2008 Movement Disorder Society

Published
2008
Full Text
View/download PDF

10. À propos de la maladie de Huntington : rôles respectifs de la famille et des professionnels de santé dans la transmission de l’information

Author

Kathy Dujardin, Luc Defebvre, Pierre Krystkowiak, Arnaud Delval, Alain Destée, and Marie Delliaux

Subjects
Neurology, Neurology (clinical)
Abstract

Resume Introduction La maladie de Huntington (MH) est une affection neurodegenerative a transmission autosomique dominante. Cependant, la facon dont l’information sur la MH se transmet au sein des familles est mal connue. Cette etude vise a preciser le role des familles et celui des professionnels de sante dans la communication de l’information sur la maladie et son risque hereditaire. Patients et methode Les donnees de 105 patients consecutifs ont ete analysees. Les patients etaient repartis en quatre categories selon la facon dont ils ont appris l’existence de la MH dans leur famille : premierement, la maladie etait connue dans la famille ; deuxiemement, la MH etait « mal connue » ; troisiemement, aucun antecedent n’etait retrouve ; quatriemement, la MH etait volontairement cachee. Resultats La majorite des patients (52 %) ne connaissaient pas le nom de la MH avant que ce diagnostic ne soit pose chez eux. Le choix des patients quant a la revelation du risque hereditaire a leur descendance est influence par la facon dont ils ont, eux-memes, appris l’existence de la maladie dans leur famille. Les patients rencontrant le plus de difficultes pour communiquer l’information sur la MH sont ceux de la deuxieme categorie (maladie « mal connue »). Discussion En depit du risque eleve de transmission, l’information sur la MH reste apparemment assez mal connue et peu transmise dans les familles concernees. Si le secret professionnel doit toujours etre respecte par les professionnels confrontes a la question de la communication de l’information aux apparentes, ces resultats soulignent, cependant, le besoin d’informer plus largement les patients sur la maladie et ses modalites de transmission. Il semble necessaire d’accompagner davantage les patients lorsqu’ils sont confrontes a la question de la transmission de l’information sur la maladie. L’efficacite de programmes specifiques d’education pourrait etre etudiee.

Published
2008
Full Text
View/download PDF

11. Rapid eye movement sleep behavior disorder in treatment-naïve Parkinson disease patients

Author

Kathy Dujardin, Luc Defebvre, Alain Duhamel, Marie Delliaux, Lucie Plomhause, Christelle Monaca Charley, and Philippe Derambure

Subjects
Male, Multiple Sleep Latency Test, Pediatrics, medicine.medical_specialty, Polysomnography, Rapid eye movement sleep, Disorders of Excessive Somnolence, REM Sleep Behavior Disorder, Cognition, Risk Factors, medicine, Humans, International Classification of Sleep Disorders, Dementia, Cognitive Dysfunction, Prospective Studies, Effects of sleep deprivation on cognitive performance, Cognitive decline, Risk factor, Aged, medicine.diagnostic_test, Parkinson Disease, General Medicine, Middle Aged, medicine.disease, Physical therapy, Female, Sleep, Psychology
Abstract

Objective Rapid eye movement (REM) sleep behavior disorder (RBD) is a risk factor for dementia in Parkinson disease (PD) patients. The objectives of our study were to prospectively evaluate the frequency of RBD in a sample of treatment-naive, newly diagnosed PD patients and compare sleep characteristics and cognition in RBD and non-RBD groups. Methods Fifty-seven newly diagnosed PD patients were consecutively recruited in a university medical center. All patients underwent two overnight polysomnography (PSG) sessions and were diagnosed with RBD according to the International Classification of Sleep Disorders, Second Revision criteria. Daytime sleepiness was measured in a multiple sleep latency test (MSLT). Cognition was assessed in a standard neuropsychologic examination. Results Seventeen PD patients (30%) met the criteria for RBD. The RBD patients and non-RBD patients did not significantly differ in mean age, gender ratio, disease duration, motor symptom subtype and severity, total sleep time, percentage of REM sleep, apnea–hypopnea index, mean oxygen saturation, and importantly cognitive performance. However, non-RBD patients had a significantly shorter mean daytime sleep latency than RBD patients (15 vs 18min, respectively; P =.014). Conclusion A high frequency of RBD was found in our sample of 57 newly diagnosed PD patients. At this stage in the disease, RBD was not found to be associated with other sleep disorders or cognitive decline. Follow-up is needed to assess the risk for developing dementia in early-stage PD patients with RBD.

Published
2013
Full Text
View/download PDF

12. Apathy in untreated early-stage Parkinson disease: relationship with other non-motor symptoms

Author

Kathy, Dujardin, Carole, Langlois, Lucie, Plomhause, Anne-Sophie, Carette, Marie, Delliaux, Alain, Duhamel, and Luc, Defebvre

Subjects
Adult, Aged, 80 and over, Male, Depressive Disorder, Depression, Apathy, Parkinson Disease, Middle Aged, Neuropsychological Tests, Disease Progression, Prevalence, Humans, Female, Fatigue, Aged
Abstract

Apathy is a frequent and disabling behavioral disorder in patients with Parkinson's disease (PD). Its prevalence in treatment-naive patients with early-stage PD has not been extensively investigated. Moreover, whether apathy is related to other non-motor symptoms in early-stage PD is unknown. Our objective was to determine the prevalence and features of apathy and associated factors in a group of treatment-naive patients with early-stage PD. Ninety-five treatment-naive patients with early-stage PD participated in the study. Apathy, depression, motor symptoms, and overall cognitive efficiency were assessed. The presence of the main non-motor symptoms was checked during a detailed clinical interview. Group comparisons were carried out to investigate the association with apathy. Eighteen patients (18.95%) were diagnosed as apathetic, and five of the latter had concomitant depression. Apathetic patients had significantly more severe motor symptoms (P 0.001) and a lower cognitive status (P = 0.032) than non-apathetic patients. When considering non-motor symptoms, apathy was significantly associated only with fatigue (P = 0.007) and anhedonia (P = 0.010), both of which were more prevalent in apathetic patients than in non-apathetic patients. In treatment-naive patients with early-stage PD, apathy was significantly associated with more severe motor symptoms and a lower cognitive status. After adjustment for these factors, apathy appeared to be a relatively isolated, independent symptom because the only other associated non-motor symptoms were fatigue and anhedonia.

Published
2014

13. Rivastigmine in apathetic but dementia and depression-free patients with Parkinson's disease: a double-blind, placebo-controlled, randomised clinical trial

Author

Pierre Krystkowiak, Jean-Philippe Azulay, David Devos, Kathy Dujardin, Alain Destée, David Maltête, Thavarak Ouk, Caroline Moreau, Alain Duhamel, Marie Delliaux, Romain Lefaucheur, Luc Defebvre, Régis Bordet, Alexandre Eusebio, Tatiana Witjas, Gilles Defer, and Alexandre Kreisler

Subjects
Adult, Male, medicine.medical_specialty, Parkinson's disease, Apathy, Phenylcarbamates, Rivastigmine, Placebo, Severity of Illness Index, Quality of life, Double-Blind Method, Internal medicine, Activities of Daily Living, Medicine, Dementia, Humans, Prospective Studies, Adverse effect, Aged, business.industry, Depression, Parkinson Disease, Middle Aged, medicine.disease, Clinical trial, Psychiatry and Mental health, Neuroprotective Agents, Physical therapy, Surgery, Female, Neurology (clinical), Cholinesterase Inhibitors, medicine.symptom, business, medicine.drug
Abstract

Background Even with optimal dopaminergic treatments, many patients with Parkinson's disease (PD) are frequently incapacitated by apathy prior to the development of dementia. We sought to establish whether rivastigmine's ability to inhibit acetyl- and butyrylcholinesterases could relieve the symptoms of apathy in dementia-free, non-depressed patients with advanced PD. Methods We performed a multicentre, parallel, double-blind, placebo-controlled, randomised clinical trial (Protocol ID: 2008-002578-36; clinicaltrials.gov reference: [NCT00767091][1]) in patients with PD with moderate to severe apathy (despite optimised dopaminergic treatment) and without dementia. Patients from five French university hospitals were randomly assigned 1:1 to rivastigmine (transdermal patch of 9.5 mg/day) or placebo for 6 months. The primary efficacy criterion was the change over time in the Lille Apathy Rating Scale (LARS) score. Finding 101 consecutive patients were screened, 31 were eligible and 16 and 14 participants were randomised into the rivastigmine and placebo groups, respectively. Compared with placebo, rivastigmine improved the LARS score (from −11.5 (−15/−7) at baseline to −20 (−25/−12) after treatment; F(1, 25)=5.2; p=0.031; adjusted size effect: −0.9). Rivastigmine also improved the caregiver burden and instrumental activities of daily living but failed to improve quality of life. No severe adverse events occurred in the rivastigmine group. Interpretation Rivastigmine may represent a new therapeutic option for moderate to severe apathy in advanced PD patients with optimised dopaminergic treatment and without depression dementia. These findings require confirmation in a larger clinical trial. Our results also confirmed that the presence of apathy can herald a pre-dementia state in PD. Registration Clinicaltrials.gov reference: [NCT00767091][1]. [1]: /lookup/external-ref?link_type=CLINTRIALGOV&access_num=NCT00767091&atom=%2Fjnnp%2F85%2F6%2F668.atom

Published
2013

14. Are Upper-Body Axial Symptoms a Feature of Early Parkinson’s Disease?

Author

David Seguy, Alain Duhamel, Marie Delliaux, David Devos, Nicolas Danel-Buhl, Thierry Perez, Kathy Dujardin, Julien Labreuche, Arnaud Delval, Céline Tard, Guillaume Baille, Luc Defebvre, Caroline Moreau, Centre de Recherche Jean-Pierre AUBERT Neurosciences et Cancer - U837 (JPArc), Université Lille Nord de France (COMUE)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, Troubles cognitifs dégénératifs et vasculaires - U 1171 (TCDV), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre d’Infection et d’Immunité de Lille - INSERM U 1019 - UMR 9017 - UMR 8204 (CIIL), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre National de la Recherche Scientifique (CNRS), Université de Lille, Lille Inflammation Research International Center - U 995 (LIRIC), Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Evaluation des technologies de santé et des pratiques médicales - ULR 2694 (METRICS), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Université de Lille, LillOA, CHU Lille, CNRS, Inserm, Troubles cognitifs dégénératifs et vasculaires - U 1171 [TCDV], Troubles cognitifs dégénératifs et vasculaires - U1171, Centre d’Infection et d’Immunité de Lille - INSERM U 1019 - UMR 9017 - UMR 8204 [CIIL], Lille Inflammation Research International Center - U 995 [LIRIC], Evaluation des technologies de santé et des pratiques médicales - ULR 2694 [METRICS], Troubles cognitifs dégénératifs et vasculaires - U 1171 - EA 1046 (TCDV), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre National de la Recherche Scientifique (CNRS)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Université de Lille-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Université de Lille, and Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Institut Pasteur de Lille

Subjects
0301 basic medicine, Pediatrics, Parkinson's disease, Pulmonology, Physiology, [SDV]Life Sciences [q-bio], lcsh:Medicine, Social Sciences, Laryngology, Dysarthria, 0302 clinical medicine, Quality of life, Medicine and Health Sciences, Psychology, Respiratory function, lcsh:Science, Movement Disorders, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, Multidisciplinary, Cognitive Neurology, Ingestion, Neurodegenerative Diseases, Parkinson Disease, Dysphagia, 3. Good health, Neurology, Physical Sciences, Anxiety, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], medicine.symptom, Research Article, medicine.medical_specialty, Patients, Materials by Structure, Cognitive Neuroscience, Amorphous Solids, Materials Science, Diaphragmatic breathing, 03 medical and health sciences, Swallowing, Neuropsychology, medicine, Speech, [SDV.NEU] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], Neuropsychological Testing, business.industry, lcsh:R, Biology and Life Sciences, Linguistics, medicine.disease, Health Care, Dyspnea, 030104 developmental biology, Otorhinolaryngology, Physical therapy, Cognitive Science, lcsh:Q, Glass, Physiological Processes, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, 030217 neurology & neurosurgery, Neuroscience
Abstract

International audience; BACKGROUND: Axial disorders are considered to appear late in the course of Parkinson's disease (PD). The associated impact on quality of life (QoL) and survival and the lack of an effective treatment mean that understanding and treating axial disorders is a key challenge. However, upper-body axial disorders (namely dysarthria, swallowing and breathing disorders) have never been prospectively assessed in early-stage PD patients.OBJECTIVE: To characterize upper-body axial symptoms and QoL in consecutive patients with early-stage PD.METHODS: We prospectively enrolled 66 consecutive patients with early-stage PD (less than 3 years of disease progression) and assessed dysarthria, dysphagia and respiratory function (relative to 36 controls) using both objective and patient-reported outcomes.RESULTS: The mean disease duration was 1.26 years and the mean UPDRS motor score was 19.4 out of 108. 74% of the patients presented slight dysarthria (primarily dysprosodia). Men appeared to be more severely affected (i.e. dysphonia). This dysfunction was strongly correlated with low swallowing speed (despite the absence of complaints about dysphagia), respiratory insufficiency and poor QoL. Videofluorography showed that oral-phase swallowing disorders affected 60% of the 31 tested patients and that pharyngeal-phase disorders affected 21%. 24% of the patients reported occasional dyspnea, which was correlated with anxiety in women but not in men. Marked diaphragmatic dysfunction was suspected in 42% of the patients (predominantly in men).CONCLUSIONS: Upper body axial symptoms were frequent in men with early-stage PD, whereas women presented worst non-motor impairments. New assessment methods are required because currently available tools do not reliably detect these upper-body axial disorders.

Published
2016
Full Text
View/download PDF

15. External globus pallidus stimulation modulates brain connectivity in Huntington's disease

Author

Luc Defebvre, David Devos, Noémie Ligot, Michel Monclus, Marie Delliaux, Serge Blond, Arnaud Delval, Philippe Peigneux, Clémence Simonin, John Van Naemen, Pierre Krystkowiak, Jérôme Yelnik, Xavier De Tiège, Christine Delmaire, Kathy Dujardin, Alain Destée, Eric Bardinet, Serge Goldman, Simon Lacroix, Service de neurologie [Amiens], CHU Amiens-Picardie, Noyaux gris centraux, Université de Lille, Sciences et Technologies-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital Erasme [Bruxelles] (ULB), Faculté de Médecine [Bruxelles] (ULB), Université libre de Bruxelles (ULB)-Université libre de Bruxelles (ULB), Neuropsychology and Functional Neuroimaging Research Unit - UR2NF, Université libre de Bruxelles (ULB), Department of Neurology et Pathologie du Mouvement (IMRT, EA2683), IFR114-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Neurology, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Services de neuroradiologie [Lille], Hôpital Roger Salengro [Lille]-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), 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), Centre de Neuro-Imagerie de Recherche (CENIR), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CIC AP-HP (pitie-Salpetriere)/inserm, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de Recherche Jean-Pierre AUBERT Neurosciences et Cancer - U1172 Inserm - U837 (JPArc), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Lille Nord de France (COMUE)-Université de Lille, Département de neurologie[Lille], Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Département de neurologie [Lille], 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), Center for NeuroImaging Research-Human MRI Neuroimaging core facility for clinical research [ICM Paris] (CENIR), Institut du Cerveau = Paris Brain Institute (ICM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-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)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-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)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Centre de Recherche Jean-Pierre AUBERT Neurosciences et Cancer - U837 (JPArc), and Université Lille Nord de France (COMUE)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille

Subjects
Male, positron emission tomography, medicine.medical_treatment, Deep Brain Stimulation, Stimulation, Cerebrovascular Circulation -- physiology, Oxygen Radioisotopes -- diagnostic use, Basal Ganglia, 0302 clinical medicine, Thalamus, Oxygen Radioisotopes, Basal ganglia, Neural Pathways, default-mode network, Image Processing, Computer-Assisted, Premovement neuronal activity, Huntington Disease -- therapy, Cerebral Cortex, Neurons, 0303 health sciences, musculoskeletal, neural, and ocular physiology, Thalamus -- physiology, Huntington's disease, Sciences bio-médicales et agricoles, Middle Aged, Neural Pathways -- physiology, 3. Good health, medicine.anatomical_structure, Globus pallidus, Huntington Disease, Neurology, Cerebral cortex, Cerebrovascular Circulation, basal ganglia, Female, Cardiology and Cardiovascular Medicine, Psychology, Adult, Deep brain stimulation, Huntington Disease -- radionuclide imaging, Brief Communication, Globus Pallidus, Neurons -- physiology, 03 medical and health sciences, Cerebral Cortex -- physiology, medicine, [INFO.INFO-IM]Computer Science [cs]/Medical Imaging, Humans, 030304 developmental biology, Nerve Net -- physiology, medicine.disease, Globus Pallidus -- physiology, nervous system diseases, nervous system, Positron-Emission Tomography, Neurology (clinical), Nerve Net, Neuroscience, 030217 neurology & neurosurgery, Basal Ganglia -- physiology
Abstract

Positron emission tomography with O-15-labeled water was used to study at rest the neurophysiological effects of bilateral external globus pallidus (GPe) deep brain stimulation in patients with Huntington's disease (HD). Five patients were compared with a control group in the on and off states of the stimulator. External globus pallidus stimulation decreased neuronal activity and modulated cerebral connectivity within the basal ganglia-thalamocortical circuitry, the sensorimotor, and the default-mode networks. These data indicate that GPe stimulation modulates functional integration in HD patients in accordance with the basal ganglia-thalamocortical circuit model., Journal Article, Research Support, Non-U.S. Gov't, SCOPUS: ar.j, info:eu-repo/semantics/published

Published
2011
Full Text
View/download PDF

16. P2‐180: New diagnostic criteria for apathy in neurodegenerative diseases

Author

Jerome Yessavage, Albert F.G. Leentjens, Barbara Bessi, Philippe Robert, Kate Dujardin, Ricardo S. Osorio, Alfonso J. Cruz Jentoft, Daniel Dachesky, Marie Delliaux, Emmanuel Mulin, Frans R.J. Verhey, Flavio Nobili, and Luis Agüera-Ortiz

Subjects
medicine.medical_specialty, Epidemiology, business.industry, Health Policy, Psychiatry and Mental health, Cellular and Molecular Neuroscience, Developmental Neuroscience, medicine, Apathy, Neurology (clinical), Geriatrics and Gerontology, medicine.symptom, Psychiatry, business
Published
2010
Full Text
View/download PDF

17. Apathy may herald cognitive decline and dementia in Parkinson's disease

Author

Kathy, Dujardin, Pascal, Sockeel, Marie, Delliaux, Alain, Destée, and Luc, Defebvre

Subjects
Male, Psychiatric Status Rating Scales, Motivation, Depression, Parkinson Disease, Middle Aged, Neuropsychological Tests, Disability Evaluation, Multivariate Analysis, Humans, Dementia, Female, Cognition Disorders, Aged
Abstract

Apathy is usually defined as a lack of motivation. It may occur as part of another disorder (notably depression and dementia) or as an isolated syndrome. In Parkinson's disease (PD), apathy is common and several studies have reported an association between this condition and more severe cognitive symptoms, such as executive dysfunction. However, this association has not been thoroughly investigated. The aim of this study (in nondepressed, nondemented PD patients) was to examine whether or not cognitive decline and/or dementia occurred more frequently in apathetic subjects than in nonapathetic subjects. Forty consecutive PD patients participated in the study (20 with apathy and 20 without). None of the subjects were either demented or depressed at the time of study entry. The patients' cognitive functions were extensively assessed twice: at study entry and after an 18-month follow-up period. At study entry, the apathetic PD patients had significantly lower global cognitive status and executive function scores than the nonapathetic subjects. After a median period of 18 months, the rate of conversion to dementia was found to be significantly higher in the apathetic group than in the nonapathetic group (8 of 20 and 1 of 20, respectively). Even in nondemented patients, the decrease over time in cognitive performance (mainly executive function but also memory impairment) was significantly greater in apathetic subjects than in nonapathetic subjects. These findings suggest that in nondemented, nondepressed PD patients, apathy may be a predictive factor for dementia and cognitive decline over time.

Published
2009

18. Cognitive complaints in Parkinson's disease: its relationship with objective cognitive decline

Author

Luc Defebvre, Catherine Thomas-Antérion, Kathy Dujardin, Alain Duhamel, Alain Destée, and Marie Delliaux

Subjects
Male, medicine.medical_specialty, Self-Assessment, Neurology, Audiology, Neuropsychological Tests, Logistic regression, Sensitivity and Specificity, Cognition, Interview, Psychological, medicine, Dementia, Humans, Cognitive decline, Psychiatry, Screening procedures, Probability, Psychiatric Status Rating Scales, Receiver operating characteristic, Cognitive disorder, Age Factors, Parkinson Disease, Middle Aged, medicine.disease, Logistic Models, ROC Curve, Multivariate Analysis, Educational Status, Female, Neurology (clinical), Psychology, Cognition Disorders
Abstract

Cognitive complaint interviews (CCI) have been shown to be useful in the early detection of dementia in elderly people. Surprisingly, CCIs are rarely used in Parkinson's disease (PD), despite a six-fold higher risk of dementia than in healthy subjects. The present study sought to determine whether a structured CCI could detect cognitive decline in PD. A validated CCI was added to the usual clinical interview for 180 PD patients. Objective cognitive status was assessed by the Mattis dementia rating scale score. The CCIs ability to detect cognitive decline in PD patients was determined using a receiver operating characteristic (ROC) curve. 58 (32.22%) patients had a significant, subjective cognitive complaint (CCI score >3). Of these, 48.27% had objective cognitive decline. Objective cognitive decline was significantly more frequent in the patients with subjective cognitive complaint. However, the ROC curve for discriminating between patients with and without objective cognitive deficits as a function of their subjective cognitive complaint had low sensitivity (0.50, 95% CI: 0.36-0.64) and moderate specificity (0.74, 95% CI: 0.69-0.84). Logistic regression incorporating the main demographical and clinical variables showed that the CCI score's discriminant power was improved by adding age and the number of years in education to the predictive model. Objective cognitive decline and dementia are more frequent among PD patients reporting a cognitive complaint than among patients not reporting a complaint. However, the CCI does not enable more accurate screening for PD-associated dementia.

Published
2009

19. Effect of external cueing on gait in Huntington's disease

Author

Arnaud, Delval, Pierre, Krystkowiak, Marie, Delliaux, Jean-Louis, Blatt, Philippe, Derambure, Alain, Destée, and Luc, Defebvre

Subjects
Adult, Male, Periodicity, Walking, Middle Aged, Cognition, Huntington Disease, Acoustic Stimulation, Humans, Ataxia, Attention, Female, Cues, Gait Disorders, Neurologic, Psychomotor Performance
Abstract

In Huntington's disease (HD) patients, gait is characterized by a timing disorder with marked intraindividual variability in temporal gait parameters (caused by the presence of both hyperkinetic and hypokinetic features). We sought to determine the influence of use of a metronome on gait parameters in patients simultaneously performing motor or cognitive tasks that required attentional resources. The objective is to evaluate the influence of rhythmic cues on gait interference during self-regulated walking and a dual task paradigm in HD. Fifteen HD patients and 15 paired controls were asked to walk and simultaneously perform another motor task (carrying a tray with four full glasses) or a cognitive task (counting backwards). We evaluated the effect of a metronome (set at 100% and 120% of the subject's self-determined cadence) in three different task conditions (gait alone, gait + motor task, gait + cognitive task). The use of auditory cues during free gait and dual tasks did not improve kinematic parameters in HD patients, in contrast to the situation for control subjects (improvement in gait speed and cadence but not stride length when the metronome was set at 120% in all conditions). HD patients have difficulty in synchronizing their footsteps with a metronome, mainly due to attentional deficits.

Published
2008

20. The Lille Apathy Rating Scale: validation of a caregiver-based version

Author

Kathy, Dujardin, Pascal, Sockeel, Marie, Delliaux, Alain, Destée, and Luc, Defebvre

Subjects
Male, Psychiatric Status Rating Scales, Emotions, Parkinson Disease, Middle Aged, Interviews as Topic, Disability Evaluation, Cognition, Caregivers, Humans, Female, Interpersonal Relations, Aged, Personality
Abstract

Apathy is reported in 16.5% to 70% of Parkinson's disease (PD) patients. Our recently developed Lille Apathy Rating Scale (LARS) has been specifically validated for patient-based assessment of apathy in PD. The aim of the present study was to validate a caregiver-based version of the LARS. Sixty consecutive PD patients and their respective caregivers participated in the study. An informant-based version of the LARS (LARS-i) was developed to rate apathy via a caregiver-based structured interview. Apathy was also assessed in a patient-based interview using the LARS and the informant- and clinician-rated versions of the Apathy Evaluation Scale (AES). Cronbach's alpha and standardized alpha coefficients were 0.872 and 0.877, respectively, and the split-half reliability was 0.901 (revealing good internal consistency). The test-retest and inter-rater reliability values were 0.960 and 0.996, respectively. Criterion-related validity (according to an independent, expert diagnosis) was good. Scores on the LARS and the LARS-i were highly correlated. However, apathy was rated significantly more severely by the caregiver than by the patient. This difference was significantly higher for demented than nondemented PD patients. The LARS-i was seen to have excellent psychometric properties and appears to be valid for use in PD with respect to the patient-based LARS and the informant- and clinician-rated versions of the AES.

Published
2008

21. Role of attentional resources on gait performance in Huntington's disease

Author

Marie Delliaux, Philippe Derambure, Kathy Dujardin, Luc Defebvre, Arnaud Delval, Pierre Krystkowiak, Alain Destée, and J.L. Blatt

Subjects
Adult, medicine.medical_specialty, Time Factors, Motor Activity, Neuropsychological Tests, Task (project management), Physical medicine and rehabilitation, Gait (human), Huntington's disease, medicine, Humans, Attention, Gait Disorders, Neurologic, Gait Disturbance, Cognition, Middle Aged, medicine.disease, Executive functions, Biomechanical Phenomena, Difficulty walking, Huntington Disease, Neurology, Physical therapy, Neurology (clinical), Cadence, Psychology, human activities
Abstract

Patients with Huntington's disease (HD) suffer from cognitive deficits with impaired executive functions, including limited attentional resources. We sought to use a dual-task paradigm to evaluate attentional demands and the ability of patients with HD to concentrate on two tasks simultaneously. We analyzed the interference effects of cognitive and motor tasks on walking in HD and the contribution of clinical symptoms to gait disturbances. Patients and controls were asked to perform either a motor task (carrying a tray with four glasses), a cognitive task (counting backwards), or no task at all while walking at their preferred speed. Kinematic spatial parameters, temporal parameters, and angular parameters related to gait were recorded in 15 patients and 15 controls by means of a videomotion analysis system. Gait instability was assessed using the stride-to-stride variability of the various gait parameters. For patients with HD, performing a concurrent cognitive task resulted in a lower gait speed (compared with free walking), with decreased cadence and stride length. However, this effect was not observed in controls. Performing a motor task did not change any kinematic gait parameters in either HD or control subjects. We found correlations between gait speed in the dual cognitive/walking task on one hand and the motor UHDRS score, cognitive status and executive function on the other. Patients with HD had greater difficulty walking while performing a concurrent cognitive task; the drain on attentional resources deteriorated walking performance.

Published
2008

22. Characteristics of apathy in Parkinson's disease

Author

Pascal Sockeel, Pierre Krystkowiak, David Devos, Marie Delliaux, Alain Destée, Kathy Dujardin, and Luc Defebvre

Subjects
Male, medicine.medical_specialty, Parkinson's disease, Multivariate analysis, Emotions, Degenerative disease, Rating scale, medicine, Prevalence, Dementia, Humans, Apathy, Psychiatry, Depression (differential diagnoses), Aged, Depression, Reproducibility of Results, Cognition, Parkinson Disease, Middle Aged, medicine.disease, Neurology, Multivariate Analysis, Female, Neurology (clinical), medicine.symptom, Psychology
Abstract

The objective of this study was to use the Lille Apathy Rating Scale to assess apathy in a large population of Parkinson's disease (PD) patients and identify several different apathy profiles. One hundred fifty-nine patients with probable PD and 58 healthy controls participated in the study. Apathy was assessed using the Lille Apathy Rating Scale. Motor, cognitive, and depressive symptoms were rated on standardized scales. Data were analyzed using linear regression and multivariate analyses of variance. Thirty-two percent of the PD patients were classified as apathetic. Apathy was more frequent in patients with dementia. The four apathy dimensions contributed differently to the overall severity of the apathetic condition. Action initiation and intellectual curiosity had a marked influence. Linear regression analysis revealed that the apathy level was mainly determined by cognitive impairment, not associated with the severity of motor symptoms, and only associated with the apathy subcomponent of the Montgomery and Asberg Depression Rating Scale. Apathy is highly prevalent in PD patients. Apathy profiles vary according to the clinical presentation of PD. The high prevalence of apathy in PD suggests the involvement of frontal-subcortical circuits. Although the neurochemical substrate of apathy remains poorly characterized, the strong link between apathy and cognitive impairment observed in several studies suggests the participation of nondopaminergic circuits.

Published
2007

27. P1.029 Apathy may be a predictive factor of dementia in Parkinson's disease

Author

C. Dernoncourt, Alain Destée, Pascal Sockeel, Luc Defebvre, Marie Delliaux, and Kathy Dujardin

Subjects
medicine.medical_specialty, Parkinson's disease, business.industry, medicine.disease, Predictive factor, Neurology, medicine, Dementia, Apathy, Neurology (clinical), Geriatrics and Gerontology, medicine.symptom, business, Psychiatry
Published
2009
Full Text
View/download PDF

28. P1.053 Effect of external cueing on gait in Huntington's disease

Author

Alain Destée, Pierre Krystkowiak, Luc Defebvre, Philippe Derambure, J.L. Blatt, Kathy Dujardin, Arnaud Delval, and Marie Delliaux

Subjects
medicine.medical_specialty, Gait (human), Physical medicine and rehabilitation, Neurology, Huntington's disease, business.industry, medicine, Neurology (clinical), Geriatrics and Gerontology, medicine.disease, business
Published
2008
Full Text
View/download PDF

29. Language processing within the striatum: Evidence from a PET correlation study in Huntington's disease

Author

Philippe Remy, Véronique Gaura, Frédéric Supiot, Pierre Renou, Anne-Catherine Bachoud-Lévi, Marc Teichmann, Marie Delliaux, Christophe Verny, and Jean-François Démonet

Subjects
Adult, Male, Striatum, Lexicon, Psycholinguistics, Text mining, Degenerative disease, Huntington's disease, Image Interpretation, Computer-Assisted, medicine, Dementia, Humans, Language, Brain Mapping, Language Tests, business.industry, Middle Aged, medicine.disease, Syntax, Corpus Striatum, Huntington Disease, nervous system, Positron-Emission Tomography, Female, Neurology (clinical), Psychology, business, Comprehension, Neuroscience, Mathematics
Abstract

The role of sub-cortical structures in language processing, and more specifically of the striatum, remains controversial. In line with psycholinguistic models stating that language processing implies both the recovery of lexical information and the application of combinatorial rules, the striatum has been claimed to be involved either in the former component or in the latter. The present study reconciles these conflicting views by showing the striatum's involvement in both language processes, depending on distinct striatal sub-regions. Using PET scanning in a model of striatal disorders, namely Huntington's disease (HD), we correlated metabolic data of 31 early stage HD patients regarding different striatal sub-regions with behavioural scores on three rule/lexicon tasks drawn from word morphology, syntax and from a non-linguistic domain, namely arithmetic. Behavioural results reflected impairment on both processing aspects, while deficits predominated on rule application. Both correlated with the left striatum but involved distinct striatal sub-regions. We suggest that the left striatum encompasses linguistic and arithmetic circuits, which differ with respect to their anatomical and functional specification, comprising ventrally located regions dedicated to rule computations and more dorsal portions pertaining to lexical devices.

30. Language processing within the striatum: evidence from a PET correlation study in Huntingtons disease.

Author

Marc Teichmann, Véronique Gaura, Jean-François Démonet, Frédéric Supiot, Marie Delliaux, Christophe Verny, Pierre Renou, Philippe Remy, and Anne-Catherine Bachoud-Lévi

Subjects
POLYETHYLENE terephthalate, HUNTINGTON disease, SYNTAX (Grammar), HUMAN behavior
Abstract

The role of sub-cortical structures in language processing, and more specifically of the striatum, remains controversial. In line with psycholinguistic models stating that language processing implies both the recovery of lexical information and the application of combinatorial rules, the striatum has been claimed to be involved either in the former component or in the latter. The present study reconciles these conflicting views by showing the striatums involvement in both language processes, depending on distinct striatal sub-regions. Using PET scanning in a model of striatal disorders, namely Huntingtons disease (HD), we correlated metabolic data of 31 early stage HD patients regarding different striatal sub-regions with behavioural scores on three rule/lexicon tasks drawn from word morphology, syntax and from a non-linguistic domain, namely arithmetic. Behavioural results reflected impairment on both processing aspects, while deficits predominated on rule application. Both correlated with the left striatum but involved distinct striatal sub-regions. We suggest that the left striatum encompasses linguistic and arithmetic circuits, which differ with respect to their anatomical and functional specification, comprising ventrally located regions dedicated to rule computations and more dorsal portions pertaining to lexical devices. [ABSTRACT FROM AUTHOR]

Published
2008
Full Text
View/download PDF

31. Human Fetal Cell Therapy in Huntington's Disease: A Randomized, Multicenter, Phase <scp>II</scp> Trial

Author

Bachoud-Lévi, Anne-Catherine, Schramm, Catherine, Remy, Philippe, Aubin, Ghislaine, Blond, Serge, Bocket, Laurence, Brugières, Pierre, Calvas, Fabienne, Calvier, Elisabeth, Cassim, François, Challine, Dominique, Gagou, Clarisse Scherer, De Langavant, Laurent Cleret, Collier, Francis, Cottencin, Olivier, David, Philippe, Damier, Philippe, Delliaux, Marie, Delmaire, Christine, Delval, Arnaud, Démonet, Jean‐François, Descamps, Philippe, Gaura, Véronique, Gohier, Bénédicte, Goldman, Serge, Haddad, Bassam, Izopet, Jacques, Jeny, Roland, Kerr-Conte, Julie, Krystowiak, Pierre, Lalanne, Christophe, Lavisse, Sonia, Lefaucheur, Jean‐Pascal, Lemoine, Laurie, Levivier, Marc, Lotterie, Jean‐Albert, Lunel‐Fabiani, Françoise, Maison, Patrick, Massager, Nicolas, Massart, Renaud, Menei, Philippe, Montero‐Menei, Claudia, Neveu, Isabelle, Parant, Olivier, Pautot, Vivien, Payoux, Pierre, Péréon, Yann, Rialland, Amandine, Rosser, Anne, Rouard, Hélène HR, Schmitz, David, Simonetta‐Moreau, Marion, Simonin, Clémence, Slama, Hichem, Sol, Jean‐Christophe, Supiot, Frédéric, Tanguy, Jean‐Yves, Tenenbaum, Liliane, Verny, Christophe, Youssov, Katia, Peschanski, Marc, Audureau, Etienne, Palfi, Stéphane, Hantraye, Philippe, Centre de référence maladie de Huntington, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), IMRB - 'NeuroPsychologie Interventionnelle' [Créteil] (U955 Inserm - UPEC), Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Université Paris sciences et lettres (PSL), Université Paris-Est Créteil Val-de-Marne - Faculté de médecine (UPEC Médecine), Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), AOM00139 and AOM04021 Direction de la Recherche Clinique, Association Française contre les Myopathies., Collaborators : on behalf the Multicentric Intracerebral Grafting in Huntington's Disease Group : Catherine Schramm, Philippe Remy, Ghislaine Aubin, Serge Blond, Laurence Bocket, Pierre Brugières, Fabienne Calvas, Elisabeth Calvier, François Cassim, Dominique Challine, Clarisse Scherer Gagou, Laurent Cleret de Langavant, Francis Collier, Olivier Cottencin, Philippe David, Philippe Damier, Marie Delliaux, Christine Delmaire, Arnaud Delval, Jean-François Démonet, Philippe Descamps, Véronique Gaura, Bénédicte Gohier, Serge Goldman, Bassam Haddad, Jacques Izopet, Roland Jeny, Julie Kerr-Conte, Pierre Krystowiak, Christophe Lalanne, Sonia Lavisse, Jean-Pascal Lefaucheur, Laurie Lemoine, Marc Levivier, Jean-Albert Lotterie, Françoise Lunel-Fabiani, Patrick Maison, Nicolas Massager, Renaud Massart, Philippe Menei, Claudia Montero-Menei, Isabelle Neveu, Olivier Parant, Vivien Pautot, Pierre Payoux, Yann Pereon, Amandine Rialland, Anne Rosser, Hélène Rouard, David Schmitz, Marion Simonetta-Moreau, Clémence Simonin, Hichem Slama, Jean-Christophe Sol, Frédéric Supiot, Jean-Yves Tanguy, Liliane Tenenbaum, Christophe Verny, Katia Youssov, Marc Peschanski, Etienne Audureau, Stéphane Palfi, Philippe Hantraye., and Montero-Menei, claudia

Subjects
0301 basic medicine, medicine.medical_specialty, Neurology, Huntington s disease, MIG-' HD, Cell- and Tissue-Based Therapy, [SDV.BC]Life Sciences [q-bio]/Cellular Biology, Disease, law.invention, 03 medical and health sciences, 0302 clinical medicine, Huntington's disease, Randomized controlled trial, law, Internal medicine, medicine, Humans, Stage (cooking), Adverse effect, [SDV.BC] Life Sciences [q-bio]/Cellular Biology, business.industry, phase 2 trial, Neurodegenerative Diseases, Sciences bio-médicales et agricoles, medicine.disease, Confidence interval, Transplantation, Huntington Disease, 030104 developmental biology, Neurology (clinical), cell therapy, business, 030217 neurology & neurosurgery
Abstract

Background\ud Huntington's disease is a rare, severe, inherited neurodegenerative disease in which we assessed the safety and efficacy of grafting human fetal ganglionic eminence intrastriatally.\ud \ud Methods\ud Patients at the early stage of the disease were enrolled in the Multicentric Intracerebral Grafting in Huntington's Disease trial, a delayed‐start phase II randomized study. After a run‐in period of 12 months, patients were randomized at month 12 to either the treatment group (transplanted at month 13–month 14) or the control group and secondarily treated 20 months later (month 33–month 34). The primary outcome was total motor score compared between both groups 20 months postrandomization (month 32). Secondary outcomes included clinical, imaging, and electrophysiological findings and a comparison of pregraft and postgraft total motor score slopes during the entire study period (month 0–month 52) regardless of the time of transplant.\ud \ud Results\ud Of 54 randomized patients, 45 were transplanted; 26 immediately (treatment) and 19 delayed (control). Mean total motor score at month 32 did not differ between groups (treated controls difference in means adjusted for M12: +2.9 [95% confidence interval, −2.8 to 8.6]; P = 0.31). Its rate of decline after transplantation was similar to that before transplantation. A total of 27 severe adverse events were recorded in the randomized patients, 10 of which were related to the transplant procedure. Improvement of procedures during the trial significantly decreased the frequency of surgical events.We found antihuman leucocytes antigen antibodies in 40% of the patients.\ud \ud Conclusion\ud No clinical benefit was found in this trial. This may have been related to graft rejection. Ectopia and high track number negatively influence the graft outcome. Procedural adjustments substantially improved surgical safety. (ClinicalTrials.gov NCT00190450.) © 2020 International Parkinson and Movement Disorder Society

Published
2020
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results